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Bleeding and thrombocytopenia are common referrals to the pediatric and adult hematology practice. The differential diagnosis encompasses a wide spectrum of entities that vary in acuity, severity, and etiology. Most will be acquired (especially in adult patients), but many can be inherited, and some may have manifestations affecting other organ systems. The first step: defining whether the symptoms and/or laboratory findings are clinically significant and warrant additional work-up, can be equally as challenging as reaching the diagnosis itself. How much bleeding is too much to be considered normal? How low of a platelet count is too low? Once the decision has been made to pursue additional studies, considering the increasing number of laboratory tests available, the diagnostic process can be complex. In this article, we outline a general approach for the evaluation of patients in whom an inherited platelet disorder is being considered. We present two clinical vignettes as introduction to the diagnostic approach to inherited platelet disorders. We describe the rationale for the different types of tests that are clinically available, their limitations, and finally the challenges that are frequently encountered in the interpretation of results. We also intend to provide some guidance on the expected phenotype in terms of severity of bleeding and/or thrombocytopenia according to the etiology of the inherited disorder. Our goal is to provide the practicing hematologist with a practical framework that is clinically applicable in their daily practice.

Belantamab mafodotin (belamaf) demonstrated deep and durable responses in patients with heavily pretreated relapsed or refractory multiple myeloma (RRMM) in DREAMM-2 (NCT03525678). Corneal events, specifically keratopathy (including superficial punctate keratopathy and/or microcyst-like epithelial changes (MECs), eye examination findings with/without symptoms), were common, consistent with reports from other antibody–drug conjugates. Given the novel nature of corneal events in RRMM management, guidelines are required for their prompt identification and appropriate management. Eye examination findings from DREAMM-2 and insights from hematology/oncology investigators and ophthalmologists, including corneal specialists, were collated and used to develop corneal event management guidelines. The following recommendations were formulated: close collaboration among hematologist/oncologists and eye care professionals is needed, in part, to provide optimal care in relation to the belamaf benefit–risk profile. Patients receiving belamaf should undergo eye examinations before and during every treatment cycle and promptly upon worsening of symptoms. Severity of corneal events should be determined based on corneal examination findings and changes in best-corrected visual acuity. Treatment decisions, including dose modifications, should be based on the most severe finding present. These guidelines are recommended for the assessment and management of belamaf-associated ocular events to help mitigate ocular risk and enable patients to continue to experience a clinical benefit with belamaf.

Antibodies and chimeric antigen receptor-engineered T cells (CAR-T) are increasingly used for cancer immunotherapy. Small molecule inhibitors targeting cellular oncoproteins and enzymes such as BCR-ABL, JAK2, Bruton tyrosine kinase, FLT3, BCL-2, IDH1, IDH2, are biomarker-driven chemotherapy-free agents approved for several major hematological malignancies. LOXO-305, asciminib, “off-the-shelf” universal CAR-T cells and BCMA-directed immunotherapeutics as well as data from clinical trials on many novel agents and regimens were updated at the 2020 American Society of Hematology (ASH) Annual Meeting. Major developments and updates for the therapy of hematological malignancies were delineated at the recent Winter Symposium and New York Oncology Forum from the Chinese American Hematologist and Oncologist Network (CAHON.org). This study summarized the latest updates on novel agents and regimens for hematological malignancies from the 2020 ASH annual meeting.

A dry tap obtained on bone marrow aspiration is considered a failure to aspirate marrow particles. However, it is often related to an underlying bone marrow pathology that hinders successful aspiration of hematopoietic cells. In this retrospective analysis, 2768 bone marrow aspirates and trephine biopsies were reviewed. Of these, 223 patients with bone marrow aspirations which yielded no marrow particles (dry tap) were included in the study. Patients in whom adequate trephine biopsy could not be obtained were excluded from the study. The final diagnosis was made by studying the trephine biopsy and correlating it with other ancillary investigations. Of the 2768 bone marrow aspirations studied, dry tap was seen in 223 (8.0%) patients. Of these, 209 (93.7%) patients had an underlying bone marrow pathology while 14 (6.3%) patients had dry tap due to faulty technique. Hematological malignancies were seen in 164 (73.5%) patients, benign hematological disorders in 33 (14.9%) patients while 4% patients had non-hematological disorders. Among hematological malignancies, leukemia (acute and chronic) was the commonest cause of a dry tap seen in 40.3% of the patients. Dry tap obtained on bone marrow aspiration should serve as a red flag for the hematologist rather than a failed procedure. In most cases, it indicates the possibility of underlying bone marrow pathology. Trephine biopsy should always be studied in such cases.

Use of granulocyte colony-stimulating factor (G-CSF) has been associated with side effects including reports of acute glomerulonephritis (GN), almost all of which have been immune complex associated. There is one prior report of pauci-immune GN in a child, but was negative for ANCA (anti-neutrophilic cytoplasmic antibodies). We describe the first case of ANCA-positive pauci-immune GN exacerbated by the use of G-CSF for peripheral blood stem cell (PBSC) donation in a patient with no prior history of vasculitis. Given the use of G-CSF in PBSC donation and neutropenias associated with various conditions, it is important that both the nephrologist and the hematologist are aware of the renal risks associated with its use.

Purpose. Presentation of clinical signs and laboratory profiles of the patients with chronic fatigue syndrome, and comparison of their symptoms with those of the patients with iron deficiency anemia. Materials and methods. Retrospective analysis of the patients’ symptoms referred to the hematologist outpatient clinic during the period between January 2016 and December 2018. Results. There were 560 patients(162 males and 398 females) referred for a primary consultation by a hematologist. Median age was 52.5 (18–94) years. The unexplained fatigue was reported by 27 (4.8%) patients (1 male and 26 females), median age — 41 (24–66) years. Diagnosis “chronic fatigue syndrome (CFS)” was confirmed based on the criteria in 17 (63%) patients. 10 patients (47%) with symptoms partially meeting the criteria were diagnosed idiopathic fatigue syndrome (SIF). Half of the patients connected the onset of the diseases with emotional trauma (family issues etc.). The prevailing complaints (>30%) were represented by: prolonged fatigue, mild memory impairment and distraction, arthralgia and insomnia. The most frequent reason to see a hematologist was fatigue and borderline changes in the blood tests. Five patients with CFS and 2 patients with SIF were known to have previously diagnosed iron deficient anemia (IDA). Median level of hemoglobin in the patients with severe fatigue and IDA was 10.7 (8.4–11.7) g/dl. Median follow up duration was 28 (6–40) months. In the observed group ( n = 23) 17% of the patients ( n = 4) showed spontaneous improvement. The rest of the patients had reported no changes. The comparison group ( n = 64) included the patients with IDA. Most of them ( n = 38) did not report fatigue as their initial symptoms (median level of hemoglobin was 9.35 (5.5–11.9) g/dl). Twenty six patients reported fatigue; median level of hemoglobin was 8.15 (5.9–11.7) g/dl. The difference between the hemoglobin levels in two groups was significant ( р < 0.05). However, there was no correlation between the level of hemoglobin and fatigue in the patients with CFS and SIF. The correlation was found in the patients with CFS and SIF between fatigue and patients’ perception. Conclusion. The main symptoms accompanying CFS are fatigue and other non-specific symptoms which are often related to patients’ emotional status. Considering CFS as a differential diagnosis when dealing with fatigue is essential.

This review aims to summarize the literature on inherited hemoglobin and coagulation disorders, focusing on sickle cell disease (SCD), thalassemia, hemophilia, and von Willebrand disease (vWD), and their impact on oral health. Clinical dental management of patients with these conditions is also reviewed. There is no evident association between caries and SCD. Despite limited available studies, there may be a possible association between caries and thalassemia, which could relate to salivary changes in immunoglobulin A and Streptococcus mutans reported in these patients. Controversial results on the caries experience of hemophilia and vWD patients are found in the literature, which are likely to reflect differences in the provided preventive dental care of these patients rather than a biological association. Current evidence does not support any association between periodontal disease and SCD. The literature on periodontal disease and thalassemia shows controversial results although some evidence suggests differences in the gingival inflammation of thalassemia patients with increased levels of inflammatory cytokines and matrix metalloproteinases, deserving further investigation. The available literature does not provide evidence of an increased risk for gingival bleeding or periodontal disease in patients with hemophilia or vWD. Dental management of these patients requires appropriate measures to prevent infection and excessive bleeding. The use of local hemostatic may often be sufficient; however, in severe bleeding disorders, systemic hemostatic or replacement therapy may be required, and procedures are recommended to be planned with the patient’s hematologist. Maintaining good oral health through preventive dentistry is important for patients with inherited hemoglobin and coagulation disorders as that may prevent the risk of infection and the need for invasive dental treatments that can result in bleeding complications.

The ongoing COVID-19 pandemic is the most trending and talked topic across the World. From its point of origin in Wuhan, China to clinical laboratory at NIH, a mere six-month-old SARS-CoV-2 virus is keeping the clinicians, and scientists busy at various fronts. However, COVID-19 is an emerging and evolving disease and each day brings in more data, new figures, and findings from the field of clinical practice. The role of hematologists has been increasingly recognized during the current pandemic because of several reasons. Most important of them are the characteristic hematological findings of COVID-19 patients that also have prognostic implications and that were not seen in other viral infections. The treatment of hematological complications in COVID-19 patients is very challenging given the critical care setting. There are interim and limited guidelines thus far due to the novelty of the disease. As this remains to be a quite fluid situation, all the appropriate medical societies including the major hematology bodies are proposing initial and interim guidelines (e.g. ASH guideline). This puts a hematologist on consult service in a dubious position where, he/she must tailor the recommendations on case to case basis. The purpose of this review is to provide the background context about the impact of COVID-19 on the blood system and to summarize the current interim guidelines to manage the associated hematological issues in COVID-19 infection.

Paroxysmal nocturnal hemoglobinuria can rarely present as cerebral ischemia and stroke due to arterial thrombosis. However, it should be considered in a young patient with bone marrow failure features, systemic thromboses, and hemolysis. The variants of paroxysmal nocturnal hemoglobinuria pose a diagnostic challenge and hence are important to recognize. We report a case of a 28-years-old female with Herlyn Werner Wunderlich Syndrome who presented with an ischemic cerebrovascular accident, pancytopenia, hemoglobinuria, and widespread abdominal thromboses suggestive of paroxysmal nocturnal hemoglobinuria. The patient was managed symptomatically and referred to a hematologist.

Successful treatment of chronic myelogenous leukemia (CML) with tyrosine kinase inhibitors (TKI) is nowadays likely to result in near-normal life expectancy. Nowadays, the treatment-free remission (TFR) is becoming more and more often a therapeutic goal for both hematologist and his patient. TKI discontinuation and maintenance of TFR is feasible in circa 40–50% of CML patients who achieved stable deep molecular response (DMR). Here, we present a patient with CML, who was switched to nilotinib due to imatinib therapy failure. After a few years of sustaining DMR nilotinib was discontinued. One year since this decision, the patient has remained in the TFR.

To prospectively quantify bleeding severity and elaborate hemorrhagic symptoms in children with 22q11.2 deletion syndrome (22q11DS) using 2 validated bleeding assessment tools (BATs), namely the Pediatric Bleeding Questionnaire and the International Society on Thrombosis and Hemostasis BAT (ISTH-BAT). We also sought to compare subjects' bleeding scores to unaffected first-degree family members.Children with 22q11DS and unaffected first-degree family members were recruited for the study. Two validated BATs were administered by a pediatric hematologist. Additional clinical and laboratory data were abstracted from patient medical records. Standard descriptive and nonparametric statistical methods were used.In total, 29 eligible subjects and controls were assessed. Median age (range) of subjects and controls was 8 (5-17) years and 38 (9-56) years, respectively. In total, 17 of 29 subjects had a positive bleeding score on ISTH-BAT compared with 1 of 29 control patients (P .0001). Median ISTH-BAT score in subjects was 3 (0-12), compared with 2 (0-6) in control patients (P = .022). Median Pediatric Bleeding Questionnaire score in subjects was 2 (-1 to 12). The most frequent bleeding symptoms reported in subjects with 22q11DS were epistaxis (69%) and bruising (52%). Eighteen subjects had been surgically challenged, and 6 were noted to have increased perioperative hemorrhage.Children with 22q11DS have increased bleeding scores compared with their first-degree unaffected relatives. The majority of the bleeding symptoms described were mucocutaneous.

Bleeding disorders constitute an important etiology of heavy menstrual bleeding in adolescents that require detailed history taking, careful planning of the laboratory evaluation, and specific management approaches. Physicians, including primary care and family practice providers, should work in coordination with a hematologist for the management of heavy menstrual bleeding in adolescents when the suspicion of a bleeding disorder is high. The two core aspects of hematology care in heavy menstrual bleeding focus on the management of iron deficiency or iron deficiency anemia and diagnostic evaluation of a bleeding disorder. School absenteeism and interruption in sports or social activities frequently occur in adolescents with heavy periods. A delay in the diagnosis of bleeding disorder interferes with a woman’s physical, social, emotional, and material quality of life. Given the consequences of HMB, healthcare providers should be able to evaluate an adolescent who presents with HMB competently. This chapter will highlight key aspects of the approach to the evaluation of bleeding disorders in adolescents with heavy menstrual bleeding.

Thrombocytopenia is often a source of concern for physicians and patients alike and is one of the commonest reasons for a hematology consultation. Through this study, we wish to ascertain the different etiologies which should be kept in mind by a hematologist when a consultation for thrombocytopenia is sought. We assessed the etiology & clinical features of thrombocytopenia seen on consultative hematology calls for patients admitted in the general ward of a tertiary care hospital. 88/277 hematology consultations taken over a course of 2 months were for thrombocytopenia. The median age of these patients was 30 years, 62.5% were female, and median platelet of 40,500/µL (1000–112000). Mild, moderate & severe thrombocytopenia was seen in 6.8%, 27.3% and 65.9% respectively. 50% of patients had a primary hematological diagnosis. Immune thrombocytopenia (ITP) was the commonest diagnosis (38.6%). Bleeding manifestations were present in 48.9% patients with 20.5% having a major bleed. One third of hematology consultations in the general ward and emergency of a tertiary care hospital are for thrombocytopenia. Almost in half, the etiology of thrombocytopenia is related to a primary hematological disorder. This information should help in decision making of use of appropriate resources.

The article presents an overview of the development of hematology service in the Republic of Tatarstan. The well-known scientist Nikolay Konstantinovich Goryaev (1875-1943), who worked in Kazan for a long time, began to develop this direction and after passing an internship in Germany proposed an improved device for calculating the blood elements known throughout the world. Adherents of Professor Goryaev continued research in the field of hematology, a blood transfusion station was organized. Professor S.I. Sherman proposed new methods of diagnosis and treatment of B12 deficiency anemia. Professor Sh.I. Ratner studied the changes in the blood picture in diseases of the abdominal cavity. The first 15 specialized hematological beds were opened in 1968 in the hospital named “Old Clinic”. The physician who treated such patients was Rakhil Sholomovna Dashevskaya, PhD. At present, hematology service is provided by three hospitals in Kazan, hematological and therapeutical beds in Naberezhnye Chelny and Nizhnekamsk, outpatient hematology service in Zelenodolsk. In recent years, the introduction of stem cell therapy has begun, and modern combined methods of chemotherapy have been introduced.

Heavy menstrual bleeding (HMB) is frequently reported by adolescents. The role of the hematologist is threefold in evaluating such patients: (1) perform a clinical and laboratory evaluation for an underlying bleeding disorder on the basis of the degree of clinical suspicion, (2) identify and manage any concomitant iron deficiency, and (3) provide input to the referring provider regarding the management of HMB, particularly for patients with identified hemostatic defects. Several clues in the menstrual history should raise suspicion for an underlying bleeding disorder, such as menses lasting >7 days, menstrual flow which soaks >5 sanitary products per day or requires product change during the night, passage of large blood clots, or failure to respond to conventional therapies. A detailed personal and family history of other bleeding symptoms should also be obtained. Iron deficiency with and without anemia is commonly found in young women with HMB. Therefore, it is important to obtain measures of hemoglobin and ferritin levels when evaluating these patients. Iron supplementation is often a key component of management in the adolescent with heavy menses and is still needed in those who have received packed red cell transfusions as a result of severe anemia. Strategies for decreasing menstrual blood flow are similar for adults and adolescents with heavy menses, with combined hormonal contraceptives recommended as first-line therapy. However, there are adolescent-specific considerations for many of these agents, and they must be incorporated into shared decision-making when selecting the most appropriate treatment.

Treatment of patients with light chain amyloidosis (AL amyloidosis) is a challenge in routine clinical practice. Although deep and durable hematologic response is crucial for the success of the therapy, in this group of patients the proper conduct of supportive care becomes particularly important. Because AL amyloidosis is an interdisciplinary disease entity, the therapeutic process should be coordinated by an interdisciplinary team consisting of a hematologist, cardiologist, nephrologist, gastroenterologist, neurologist and clinical dietician. In this paper, we present a strategy for treating a patient with a newly diagnosed systemic intermediate-risk AL amyloidosis with cardiac involvement in the Department of Hematology of the Institute of Hematology and Transfusion Medicine in Warsaw, in which, apart of standard chemotherapy, doxycycline was used.

BackgroundDrug-induced thrombocytopenia (DITP), which also includes thrombocytopenia induced by beverages, foods, and herbal remedies, is an important clinical problem for haematologists. Drug-induced thrombocytopenia is often misdiagnosed as immune thrombocytopenic purpura with resulting inappropriate treatment. Immune thrombocytopenic purpura (ITP) is a clinical disorder that leads to easy bruising (purpura), excessive bleeding or extravasation of blood from capillaries into skin and mucous membranes (petechiae). The bleeding tendency is due to decreased number of circulating platelets (thrombocytopenia). There is production of antibodies against the platelets by the patient’s immune system. This case report was conducted to introduce the effectiveness of freshly donated blood and steroids on patients with immune thrombocytopenic purpura (ITP) after ingestion of herbal drugs.Case descriptionDT was a 30-year old female who presented with bleeding per vagina, gum bleeding and weakness. The patient did not have any systemic disease that would cause any spontaneous hemorrhage. The patient was referred to a hematologist urgently and her thrombocyte count was found to be 2000/ìL. Other test results were in normal range. Full blood count revealed severe thrombocytopenia. Freshly donated whole blood was given to the patient and then the changes in her general condition were analyzed, as well as the blood test results.ConclusionIn the absence of platelet concentrate especially in rural settings and resource-poor countries, freshly donated whole blood can be used in the management of a case of severe thrombocytopenia from ITP.

While patients with immune thrombocytopenia (ITP) and low platelet counts are at risk for bleeding, they are not protected against arterial and venous thrombotic events. Frequently, hematologists are asked to consult on a patient with ITP requiring an antiplatelet (AP) agent or anticoagulant (AC). No direct evidence exists to guide hematologists in weighing the risk of thrombosis against the risk of bleeding in patients with ITP. Therefore, we performed a survey to determine the preferred management of AP/AC therapy in ITP patients. The survey described hypothetical patient scenarios and asked respondents to recommend a minimum platelet count for initiation of AP/AC therapy. We surveyed both hematologists with an international reputation in treatment of ITP (n = 48) and also general hematologist–oncologists in Oklahoma (n = 97). Response rates were 38/48 (79%) for the ITP specialists and 46/97 (47%) for general hematologist–oncologists. Overall, recommended platelet thresholds for antithrombotic therapy were similar between ITP specialists and general hematologist–oncologists. Although both groups recommended a minimum platelet count of 50 × 109/L for AP and AC therapy in most scenarios, there was great variability in individual practice patterns among respondents. This study highlights the need for studies of patients with ITP who require AP/AC therapy to provide high-quality evidence for establishing optimal management strategies.

Multiple myeloma is a hematological malignancy, which sometimes creates difficulties in diagnosis, based on the presence of monoclonal protein in serum/urine and plasmocyte infiltration in the bone marrow, and on the other hand, synchronous cancers are also a diagnostic challenge. We present a case report of a patient with concurrent breast cancer and multiple myeloma. A 68-year-old woman was admitted to the hospital with diagnosis of left breast cancer in first stage of the disease. In the past, she had had several episodes of thrombocytopenia, leucocytosis, and mild anaemia, which were followed by hematologist in outpatient setting. She was operated and started adjuvant chemotherapy. During treatment, episodes of hematological abnormalities were observed. After completion of the chemotherapy for breast cancer, the patient was observed and short time after that multiple myeloma was diagnosed as a synchronous cancer. Patient was first treated for breast cancer, then subsequently for multiple myeloma (2 lines therapy: CTD and VMP). We describe diagnostic problems with multiple myeloma; however, they could be caused by curation of breast cancer, which might have supressed the proliferation of plasmocytes and could delay the diagnosis. All symptoms of multiple myeloma were interpreted as a secondary effect of chemotherapy. Review of the literature shows the clinical approaches in such situations.

In the article the clinical observation of a rare combination of ulcerative colitis with von Willebrand’s disease in 33-year-old patient. Comorbidity require specific types of the disease, taking into account the peculiarities of its course, monitoring the main hematological parameters for the prevention of massive blood loss during the treatment of ulcerative colitis, helped damage colonic mucosa (Activity II by Schroeder) and the possibility of spontaneous gastrointestinal bleeding. The article demonstrated features of the diagnostic and treatment process, described diagnosis, assessment of severity of main and concomitant diseases, as well as the dynamics of the patient’s condition during treatment. The choice of treatment tactics was complicated because of the multi-directional impact of both diseases on hemostasis, but the definition of the main hematological parameters allowed to forecast a stable course and not to use drug therapy of von Willebrand disease. The organization of the medical process in ulcerative colitis, associated with von Willebrand’s disease, a comprehensive approach that includes Standard treatment for ulcerative colitis srednetyazhёlogo flow was used: systemic glucocorticosteroids mesalazine sulfosalazin locally and orally, as well as other products in accordance with current clinical guidelines. Close cooperation between two specialists (gastroenterologist and hematologist) in charge of one patient allowed to achieve clinical and laboratory remission

Of all the cancers, chronic myeloid leukaemia (CML) has witnessed the most rapid evolution of the therapeutic milieu in recent decades. The introduction of tyrosine kinase inhibitors (TKIs) as a therapeutic option has profoundly changed patient experience and outcome. The availability of multiple new highly effective therapies has increasingly underscored the importance of a good understanding of the underlying pathophysiological basis in CML, as well as patient-specific factors in choosing the right treatment for every individual. The treatment of CML has migrated in many jurisdictions from the office of a highly specialized malignant hematologist to the general hematologist or even a general practitioner. The goal of this review is to offer an overview of the modern approach to the treatment of CML, with an emphasis on chronic phase (CP) CML, including both TKI-based therapies such as imatinib, dasatinib, nilotinib, bosutinib and ponatinib, and non-TKI medications, such as omacetaxine. We discuss evidence behind each drug, most common and material adverse reactions and outline how this information can be used in selecting the right drug for the right patient. We also discuss evidence as it relates to other therapies, including stem cell transplant (SCT), and patients in accelerated (AP) and blastic phase (BP).

To evaluate differences between pediatricians and internists in the practice of and barriers to advance care planning (ACP) for adolescent patients with cancer.A self-reported questionnaire was administered to assess the practice of ACP, advance directives, and barriers to ACP for adolescent patients with cancer. All 3392 Japanese board-certified hematologists were surveyed, and 600 hematologists (227 pediatricians, 373 internists) who take care of adolescent patients with cancer with decision-making capacity were analyzed.If a patient's prognosis for survival was3 months, pediatricians were significantly less likely to discuss ACP with their patients than internists, including discussions regarding the patient's medical condition (59% vs 70%), the patient's understanding of his/her medical condition (55% vs 66%), do not attempt resuscitation orders (17% vs 24%), and ventilator treatment if the patient's condition worsened (19% vs 25%). More than 75% of hematologists (both pediatricians and internists) discussed all ACP topics with patients' families. Similarly, with regard to advance directives, pediatricians were less likely than internists to discuss cardiopulmonary resuscitation (24% vs 47%) and the use of ventilators (31% vs 51%), vasopressors (24% vs 42%), and antibiotics (21% vs 31%) with their patients. Both pediatricians and internists discussed these issues more often with patients' families than with patients, especially cardiopulmonary resuscitation (98%) as well as the use of ventilators (98%) and vasopressors (91%).Pediatricians were less likely than internists to discuss ACP and advance directives with patients, and both pediatricians and internists tended to discuss ACP and advance directives more often with patients' families.

When faced with a life‐threatening non‐cancerous blood disorder, the term “benign” is a misnomer. Devastating diseases like catastrophic antiphospholipid antibody syndrome, acquired hemophilia, and severe immune thrombocytopenia present a challenge to the hematologist. They are often difficult to treat and lack appropriately powered, unbiased evidence to support management. Moreover, the label “benign” does a disservice as it subconsciously triggers discrepancies in prioritization for the care provider, the system, the patient and his/her family. Despite our progressive advances in non‐malignant hematology, there remain many knowledge and care gaps that can be effectively addressed by more international collaboration, more clinical and research infrastructure and more expertly trained clinicians. To highlight the need, is it time to reconsider the term “benign” hematology?

Disorders of fibrinolysis result in bleeding of varying severity with no pathognomonic features distinguishing one disorder from the other. Additionally, these hyperfibrinolytic states are rare and challenging to diagnose due to lack of standardized, reproducible assays which are not widely available. A hematologist should have a high index of suspicion for fibrinolytic disorders if there is delayed bleeding after surgery or trauma and/or heavy menstrual bleeding wherein tier-one evaluation for common bleeding disorders has not yielded conclusive results. Global assays may prove useful in these rare scenarios. Treatment relies on the use of antifibrinolytic therapy and as needed fresh frozen plasma for severe bleeding presentations.

With the increase in number of clinical centers focused on vascular anomalies, it is evident that a broad, interdisciplinary approach is optimal. Hematology/oncology (H/O) is an integral specialty for a vascular anomaly team, as many of the clinical sequelae that are encountered are within the scope of practice of H/O. Nonvascular oncologic diagnoses (such as certain solid cancers) are part of the differential diagnosis for vascular anomalies and malformations, experience with chemotherapy and developmental therapeutics, expertise with the management of coagulopathy and thromboses, and workup of anemia and thrombocytopenia are all expectations for H/O subspecialists. For some institutions, the incorporation of patients with vascular anomalies into the H/O program or a provider’s clinical workflow can be challenging. The thoughtful consideration of the hematologist/oncologist’s role in the context of the clinical infrastructure and available services at each center will promote optimal patient care and clinician well-being.

Introduction. Glanzmann’s thrombasthenia is a rare clotting disorder caused by impaired platelet function. Lack of awareness of the appropriate management of rare medical conditions may lead to patient dissatisfaction and potentially poor treatment outcome. Case Report. A 78-year-old male with a history of Glanzmann’s thrombasthenia was admitted to the trauma service following a fall in which he sustained a facial laceration as well as maxillary sinus and nasal fractures. He received DDAVP 20 mcg and tranexamic acid upon presentation to the emergency department (ED). In the ED, the patient requested administration of platelet transfusion but was refused due to a normal platelet count. During the course of his hospital stay, he complained of epistaxis and was noted to have a downtrending hemoglobin from 11.0 g/dl to 9.0 g/dl. The patient and his family were not comfortable when the discharge plan was finalized and demanded platelet transfusion (due to history of needing platelets in association with injuries or procedures in the past) was refused by the primary team as they continued to state that his platelet count is normal. On hospital day 3, hematology was consulted as the patient and his family were extremely angry and hematology recommended platelet transfusion. Further clinical information was not available as the patient was transferred to another facility per family request as they wanted to be at a center which had the patient’s primary hematologist. Discussion. A delay in specialist consultation resulted in patient dissatisfaction and extended the length of stay. Patients with rare medical conditions and potential for major complications should be managed aggressively with appropriate specialist consultation to promote patient satisfaction and improve the overall quality of care. This case shows that as physicians it our duty to listen to our patient’s concerns and involve them in the medical decision-making to provide optimal patient-centered care.

Importance With 80% of childhood cancer survivors (CCS) alive 30 years after diagnosis, preventable causes of death, such as cardiovascular disease resulting from initial cancer therapy, becomes an important metric. This leads to a more pronounced role for cardiologists in the care of CCS. Observations While routine cardiovascular screening has been traditionally performed by the hematologist/oncologist or primary care provider, our understanding of cardiovascular disease in CCS has advanced. The measurement of left ventricular ejection fraction (LVEF) can now be complemented with additional assessments of strain, LV mass, right ventricular function, diastolic function, valve function, the pericardium, coronary perfusion, and biomarkers. Risk factor modification, prophylaxis, and timing of treatment are also critical. Conclusions and Relevance Early cardiovascular screening and treatment in asymptomatic CCS can be nuanced and complex. As a result, there is a renewed opportunity for the cardiologist to play an integral role in the care of CCS. Key Points Question/Purpose: Review cardiovascular disease and the role of the cardiologist in the care of asymptomatic childhood cancer survivors (CCS). Findings: Cardiovascular care in CCS benefits from a multi-faceted approach that does not overly rely on LVEF. Meaning: Adequate screening and treatment of cardiovascular disease in asymptomatic CCS may often be optimized by the involvement of a cardiologist.

Lymphomas are one of the most common malignant entries across all populations. Originating most commonly from the lymph nodes, extranodal lymphomas, including those originating from the connective tissue capsule of the internal organs, are rare entries. Herein we present a case report of a 78-year-old male patient presenting with a palpable mass in the region of the left kidney. Ultrasound and computer tomography revealed a mass engulfing the kidney. Nephrectomy was performed, with the histopathological investigation revealing a non-Hodgkin lymphoma originating from the renal capsule, with infiltration into the adjacent tissues. The patient was referred to a hematologist for treatment and one and a half years later, following two negative bone marrow biopsies, the patient is alive and disease-free.

BACKGROUND Ensuring that patients with hematologic malignancies have an accurate understanding of their likelihood of cure is important for informed decision making. In a multicenter, longitudinal study, the authors examined discordance in patients' perception of their chance of cure versus that of their hematologists, whether patient-hematologist discordance changed after a consultation with a hematologist, and factors associated with persistent discordance. METHODS Before and after consultation with a hematologist, patients were asked about their perceived chance of cure (options were

Leukemia creates abnormal and immature cells in the bone marrow which cannot fight infection effectively. To reduce the death and severe suffering, effective early diagnosis and screening are needed. Leukemia is diagnosed with complete blood count and bone marrow examination. The manual diagnosing of leukemia needs special training and it is not accurate when done by non-experts. Hence an automated system is necessary for diagnosing and classifying the subtypes of leukemiaearlier for proper treatment using blood microscopic images. This automated system will increase the accuracy of all subtypes and it is useful in the rural areas where hematologist’s experts are less. Many researchers have proposed different methods for diagnosing the leukemia. This paper gives the review of various methods with improved accuracy and their results are compared.

Hematology is the study of diseases and disorders causing irregularities in blood cells. Hematology is used to determine the irregularities in the blood cell containing different parasites ranging from mild to severe symptoms. Malaria has been found to be the 9th leading cause of death in the country. Malaria is caused by a bite of an Anopheles mosquito, an infected mosquito carrying a virus called Plasmodium parasite that will then be released onto a person's blood stream. The general objective of this study is to detect blood parasite from sample blood smears. This study is obtrusive and the device requires a blood sample to be tested, the researchers let expert medical doctors and laboratorians to take the blood from the patients. In hope of helping the telemedicine in the Philippines the device is able to capture images from the microscope and designed an algorithm that will process the captured images stitching it into one whole image. In addition, the device can output if the blood sample is positive or negative based on the dominant color seen from the images. The prototype is designed to replace the analytics of a skilled hematologist in rural cases whereas shortage in experts is a major problem.

The article describes the case of the primary stomach lymphoma of the stomach in a 29-year-old man. The literature data on the etiology, diagnosis and morphological characteristics of the primary lymphomas of the stomach are presented, the questions of treatment tactics, variants of cytostatic therapy schemes are touched upon. In the described clinical example, the patient’s medical history is given: from the age of 16 the chronic gastroduodenitis is diagnosed, the patient periodically took omeprazole with a positive effect, there is no evidence of infection with Hp and there is no eradication. In April 2016, he noted the deterioration of health in the form of the appearance of discomfort in the epigastric region. In April 2016, an EHDS was performed at the outpatient stage, where an antral tumor of the stomach was detected, and a biopsy of the formation was taken. Histological examination of atypical cells was not revealed, and in connection with this, in May 2016, he was hospitalized at the A.M. Burnazyan for additional examination. The article presents data of laboratory and instrumental examination, the results of EGDS, CT of thoracic and abdominal cavities, cytological, immunohistochemical, and cytogenetic studies. The patient was given three courses of cytostatic therapy. The first course of cytostatic therapy was started in June 2016, the patient transferred it satisfactorily, in the post-cytostatic period there was a leukopenia of 2,5 thousand cells/μl, against which the viral lesion of the tongue mucosa developed, and the therapy with virolex was performed with positive dynamics. The EGD control showed marked positive dynamics. The second and third courses were conducted in July-August 2016, treatment was satisfactory. In September 2016 he was discharged for dynamic observation by a hematologist. The article draws the attention of medical practitioners to the fact that, in the absence of specific clinical manifestations, the basis for the diagnosis of lymphoma in this clinical example is the timely conduct of an endoscopic study with histological, immunohistochemical and cytogenetic study of the biopsy.

The article presents literature data and a clinical case on the rare Imerslund-Grasbeck syndrome. The pathogenetic foundations, the features of the clinical picture, course and treatment of this syndrome in children are discussed. Own clinical observation of the syndrome of Imerslund-Grasbeck in a 8 years girl is presented. We presented a summary table with described in the literature of mutations in different ethnic groups. The authors indicated the need for molecular genetic studies of genes CUBN and AMN in children with the combination of proteinuria and B12-deficiency anemia to diagnose the Imerslund-Grasbeck syndrome. Patients with the Imerslund-Grasbeck syndrome require lifelong replacement therapy with cobalamin, observation by a nephrologist and a hematologist.

Obstetrical hematology represents challenges not only for the patient, but also for her progeny. In particular, bleeding disorders, both congenital and acquired, not only present problems both for delivery and in the immediate postpartum period, but also may have significant implications for the fetus and neonate. Women with congenital bleeding disorders or who are carriers of X-linked or autosomal disorders should be counseled prior to conception so that pregnancy can be safely undertaken with careful preparation. A treatment plan should be set up by a specialized care team that includes the hematologist, obstetrician, and anesthesiologist; the patient should be followed closely during pregnancy, through delivery, and in the immediate postpartum period. Acquired disorders of hemostasis that occur with pregnancy may present particular diagnostic difficulties and require rapid diagnosis and management.

Patients with advanced hematological malignancies are less likely to be referred to specialist palliative care services compared with patients having solid tumors. It has been reported that one of the most important reasons for the lack of referral is difficulties in the prognostication of terminally ill patients with hematologic malignancies. The study objective was to evaluate the predictive accuracy of the Palliative Prognostic Index (PPI) and the prognostic model developed by Kripp et al in hospitalized patients under the care of a hematologist. Using clinical charts, we retrospectively calculated the above scores. We reviewed the records of 114 patients admitted to the hematology ward. The inclusion criterion was patient with disease considered incurable using standard treatments. The prognostic models were assessed according to the original reports. Using PPI cutoff points of 2 and 4, we divided the patients into 3 groups of significantly different survival times ( P < .01). Moreover, we confirmed the usefulness of predicting survival

Study Objective An IV iron rapid referral pathway (IVIRRP) was established as an electronic-medical record (EMR)-based protocol in collaboration with Hematology, to expedite delivery of IV iron to anemic patients undergoing benign hysterectomy. We evaluated the effect of the IVIRRP on the rates of preoperative anemia, referrals for IV iron, rates of transfusion, and time from referral to infusion. Design Quality improvement intervention study Setting Academic medical center Patients or Participants 278 patients in the pre- and 250 patients in the post-intervention cohort. Interventions Gynecologic surgeons were provided with guidelines for implementing the IVIRRP. Referrals were reviewed by a hematologist who coordinated IV iron infusion. Measurements and Main Results Baseline anemia, defined as a hemoglobin (Hb) Conclusion Implementation of an EMR- based, multidisciplinary, rapid referral protocol for anemic patients pre-operatively increases the rate of referral for IV iron, expedites delivery of IV iron, and results in increased pre-operative hemoglobin and decreased rates of transfusion.

Introduction: According to the World Health Organization (WHO), COVID-19 has become a Public Health Emergency of International Concern (PHEIC). Understanding patients' hematologic findings in SARS-CoV-2 infection is essential to doing their prognosis, so adjusting care and improving outcomes. Objective: In this review, we aim at summarizing changes in the hematopoietic system and hemostasis that occur in SARS-CoV-2 infected patients. Findings: COVID-19 infection is often associated with laboratory hematologic features that can have important clinical implications. Careful revision of baseline hematologic data at diagnosis can predict the severity of illness and help clinicians tailoring the approach and management of patients whose condition can be guarded or critical. The levels of hematologic markers like D-dimer, procalcitonin, C-reactive protein, viral load, inflammatory cytokines, differential blood cell count, and peripheral smear are fundamental for the prognosis. Studies have also shown an association between some of these markers and severe COVID-19 infection requiring admission to the intensive care unit or complicated by acute respiratory distress syndrome (ARDS). Since, so far, a vaccine is not available, prevention of the infection is based on the avoiding people affected and the spreading of the virus; the treatment, in the absence of an effective antiviral agent, is symptomatic, and, in addition to oxygen support, finds in the anti-inflammatory drugs and anticoagulation fundamental therapeutic lines. According to the American Society of Hematology (ASH), all hospitalized patients with COVID-19 should receive pharmacologic thromboprophylaxis with LMWH.

Background Scurvy is a rare entity in developed countries and the diagnosis may often be delayed resulting in unnecessary investigations and/or potentially severe complications. A recent increase in the number of patients diagnosed with scurvy in our hematology clinics indicated the need to review the literature on the diagnosis and optimal management of similar patients. Methods We conducted a retrospective chart review of patients referred to hematology at our tertiary care centre between 2010 and 2018, who were ultimately diagnosed with scurvy. Data collected from electronic medical records included baseline characteristics, clinical features on presentation, bloodwork results from initial consultation, treatment plan as well as response to treatment. Findings Twenty-two adults patient had a diagnosis of scurvy with a mean vitamin C level of 6 μmol/L. Iron deficiency anemia (54%) and gastrointestinal disorders (54%) were the most common comorbidities noted in our cohort. Proton-pump inhibitors use was noted in 54% of patients. Bleeding (45%) and bruising (45%) were the most commonly reported clinical features. Eleven patients received oral supplementation, five had intravenous (IV) vitamin C and six were not treated. Two patients required a transition from oral to IV supplementation. Vitamin C dosing ranged between 250 and 2000 mg and the frequency varied from daily for oral therapy to every few weeks or months for IV. Interpretation Awareness of scurvy and its associated risk factors and clinical presentation is important in the evaluation of a patient with bleeding tendency. Treatment plan should be individualized, and a careful review of patients' diet, medial history and medications is warranted.

Venous thromboembolism (VTE), which includes both deep venous thrombosis and pulmonary embolism, is a common and potentially fatal condition. Medical inpatients are at high risk for VTE because of immobility as well as acute and chronic illness. Several randomized trials demonstrated that chemoprophylaxis, or low-dose anticoagulation, prevents VTE in selected medical inpatients. The 2018 American Society of Hematology clinical practice guideline on prophylaxis for hospitalized and nonhospitalized medical patients conditionally recommends chemoprophylaxis for non-critically ill medical inpatients, leaving much to the discretion of the treating physician. Here, 2 experts, a hematologist and a hospitalist, reflect on the care of a woman hospitalized with a rheumatologic disorder. They consider the risks and benefits of chemoprophylaxis, discuss VTE risk stratification, and recommend which patients should receive chemoprophylaxis and with which agents.

Thrombocytopenia during pregnancy presents unique challenges for the hematologist. Obstetricians generally manage many of the pregnancy-specific etiologies, ranging from the benign (gestational thrombocytopenia) to the life-threatening (preeclampsia; hemolysis, elevated liver enzymes and low platelets syndrome; and acute fatty liver of pregnancy). However, hematologists may be consulted for atypical and severe presentations and to help manage non-pregnancy specific etiologies, including immune thrombocytopenia, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome and antiphospholipid syndrome, among others, in which maternal and fetal risks must be considered. This review provides a general approach to the diagnosis and management of thrombocytopenia in pregnancy for the consulting hematologist.

This review summarizes - with the practicing hematologist in mind - the methods used to determine measurable residual disease (MRD) in everyday practice with some future perspectives, and the current knowledge about the prognostic impact of MRD on outcome in acute myeloid leukemia (AML), excluding acute promyelocytic leukemia. Possible implications for choice of MRD method, timing of MRD monitoring, and guidance of therapy are discussed in general and in some detail for certain types of leukemia with specific molecular markers to monitor, including core binding factor (CBF)-leukemias and NPM1-mutated leukemias.

Purpose of review We present an overview of the clinically available immunotherapeutic platforms centered on T cell lymphocytes for the treatment of hematological malignancies with a special focus on the advances achieved since 2017. Recent findings The most promising strategies of T cell therapies are based on the infusion of unmanipulated donor lymphocytes, antigen-specific T cells, ex-vivo genetically modified T cells or in-vivo manipulation of T cells using antibodies (bispecific and checkpoint inhibitors). To select the most appropriate option for a specific patient, is important to understand not only each platform-specific biological mechanisms, clinical activity and toxicities but also their intrinsic differences. In this extremely fast-growing field, to keep track of recent advances is a hard and challenging task for a hematologist. Therefore, we aimed to provide a global integrative review of T-cell-based immunotherapies, essential to ascertain their optimal place and timing in our daily clinical practice. Summary We highlight recent advances and discuss future directions of T-cell-based immunotherapies entering the common clinical practice in hematology.

OBJECTIVES: Incidental isolated mild to moderate thrombocytopenia is a frequent laboratory finding prompting a referral to pediatric hematology-oncology. We tested the hypothesis that patients with isolated asymptomatic mild thrombocytopenia would not progress to require an intervention from a pediatric hematologist–oncologist. METHODS: This is a 5-year retrospective review of 113 patients referred to pediatric hematology–oncology for isolated thrombocytopenia. Initial, lowest, and current platelet counts along with clinical course and need for interventions were recorded. Thrombocytopenia was categorized as mild (platelet count: 101–140 × 103/μL), moderate (platelet count: 51–100 × 103/μL), severe (platelet count: 21–50 × 103/μL), and very severe (platelet count: ≤20 × 103/μL). RESULTS: Eight of 48 patients (17%) referred for initial mild isolated thrombocytopenia progressed to moderate thrombocytopenia at 1 visit. At present, 2 of these patients have moderate thrombocytopenia, 17 remain with mild thrombocytopenia, and 29 patients have resolved thrombocytopenia. Nine of 65 patients (14%) referred for moderate thrombocytopenia progressed to severe or very severe thrombocytopenia on 1 occasion. At present, no patients have severe thrombocytopenia, 18 remain with moderate thrombocytopenia, 14 improved to mild thrombocytopenia, and 33 have resolved thrombocytopenia. Only 3 patients required interventions from a hematologist, whereas 10 patients required therapy from other subspecialties. CONCLUSIONS: We only identified 3 patients (3%) with mild to moderate thrombocytopenia who required an intervention from a hematologist to improve platelet counts. Patients with isolated mild thrombocytopenia with a normal bleeding history and physical examination findings frequently have normalized their platelet counts within 1 month.

Bone marrow necrosis (BMN) is a condition that can be difficult to diagnose, requiring a hematologist experienced in bone marrow morphology. This diagnostic challenge should alert the clinician of a severe disease or a possible underlying malignancy, either hematological or a solid tumor. We describe the concomitant presence of a primary bone marrow lymphoma (diffuse large B-cell lymphoma-DLBCL), along with an extensive BMN in an HIV patient for the first time in a living individual. HIV infection, BMN and DLBCL presented a multifactorial crossword of molecular events underlying the complex pathophysiology. The exact precipitating pathophysiological events resulting in BMN remain obscure and provide their clear impact for future research. The present report is instructive and also contains a critical review of the literature related to the case presented.

To determine the referral patterns and etiology of iron deficiency anemia (IDA) at an academic hematology center in northeast Mexico.We included all consecutive outpatients older than 16 years, non-pregnant, with IDA diagnosed in the Hematology Service of the Dr. José E. González University Hospital between January 2012 and May 2017. Appropriate data were collected retrospectively from the electronic medical record. Data regarding first medical contact (primary care physician or hematologist) were compared.One hundred fifty-three patients were included in this study. The median age was 43 years (interquartile range, 35-51) and 85.6% were female; 128 (83.7%) patients were seen by a primary care physician before our evaluation. Abnormal uterine bleeding (AUB) was the cause of IDA in 76 patients (49.6%), gastrointestinal bleeding (GIB) in 31 (20.2%), H. pylori infection in 12 (7.8%), urinary tract bleeding in three (1.9%) and malabsorption-syndrome in two (1.3%). The etiology remained unknown in 29 (18.9%). The p value was0.05 between groups according to the first medical contact, including frequency of at least one sign or symptom of IDA, previous use of iron supplementation and blood transfusion, comorbidities, complete blood count at diagnosis, and resolution rates of anemia.The majority of our IDA patients were referred by another physician. Nearly half of the patients with IDA had AUB. IDA remains a diagnostic challenge for first contact physicians requiring a targeted educational intervention to improve IDA awareness and diagnostic skills.

This year we celebrate Blood's 70th year of publication. Created from the partnership of the book publisher Henry M. Stratton and the prominent hematologist Dr William Dameshek of Tufts School of Medicine, Blood has published many papers describing major advances in the science and clinical practice of hematology. Blood's founding antedated that of the American Society of Hematology (ASH) by more than 11 years and Stratton and Dameshek helped galvanize support for the creation of ASH. In this review, I place the birth of Blood in the context of the history of hematology before 1946, emphasizing the American experience from which it emerged, and focusing on research conducted during World War II. I also provide a few milestones along Blood's 70 years of publication, including: the growth in Blood's publications, the evolution of its appearance, the countries of submission of Blood papers, current subscriptions to Blood, and the evolution of topics reported in Blood's papers. The latter provides a snapshot of the evolution of hematology as a scientific and clinical discipline and the introduction of new technology to study blood and bone marrow. Detailed descriptions of the landmark discoveries reported in Blood will appear in later papers celebrating Blood's birthday authored by past Editors-in-Chief.

The increasing use of immunophenotypic and molecular analysis in the routine evaluation of patients with lymphocytosis, lymphadenopathy, or other hematologic disorders has led to the identification of unexpected small clonal lymphoid populations. These clones, sometimes with disease-specific markers, such as the t(14;18), are especially challenging for the clinician because of their unknown biologic potential and uncertain clinical behavior. Study of these early lymphoid lesions is providing important clues to the process of lymphomagenesis, and may provide the rationale for preemptive therapy in the future. More and more, the hematologist/oncologist is consulted regarding otherwise healthy individuals with lymphadenopathy and/or lymphocytosis, and pathology reports that confound the referring internist or surgeon. The report does not name a malignant lymphoproliferative disorder, but is not completely “normal”. Does the patient have a benign or malignant condition? How should they be evaluated? Is treatment indicated? These patients prove challenging for the consulting hematologist as well as the referring physician. In this review, we will focus on some of these scenarios and attempt to provide guidance for their management.

In 1963, Jean Bernard, a French hematologist, opened a new chapter in hematology called geographic hematology (‘Hématologie Géographique’). He distinguished two research avenues. One dealt with the differences between the various populations (ethnic hematology), the other with various environmental factors (environmental hematology). In recent years, focus has been put on analyzing the genetic susceptibility in cancer and hematological malignancies, particularly in childhood acute lymphoblastic leukemia, using specific gene or (genome-wide association study) approach. However, almost 30 years ago, it was suggested by a few workers that chromosomal abnormalities observed in leukemia could have a geographic and/or ethnic distribution. In this review, we analyze the literature on chromosomal and molecular abnormalities in several types of leukemia.

Erythrocytosis is frequently encountered as an incidental abnormality on laboratory testing that reveals persistent elevation of the hematocrit level (>52% in men and >48% in women). In many cases, erythrocytosis is the manifestation of an underlying cardiopulmonary process, drug-induced due to androgens, or secondary to smoking, rather than a primary bone marrow disorder such as polycythemia vera. A systematic approach to the clinical and laboratory evaluation of each patient is indicated to consider diverse differential diagnosis possibilities and to identify the underlying etiology of erythrocytosis in order to formulate appropriate subspecialist referral and management plans. A thorough medical history and meticulous physical examination supplemented by a focused initial laboratory evaluation will enable the general practitioner to ascertain the etiology of erythrocytosis in the majority of cases. Patients with clinical and laboratory features suggestive of polycythemia vera and those patients without an apparent underlying condition known to cause erythrocytosis benefit from early referral to a hematologist for further specialized diagnostic evaluation and therapy considerations.