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A hematologist receives a call from a maternal-fetal medicine (MFM) physician about a previously healthy patient who became ill at 25 weeks’ gestation. Her mental status is deteriorating. There are signs of fetal distress. Platelet count and hemoglobin are falling. The MFM physician is considering the hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. For the hematologist, everything seems unfamiliar. Our goal is to provide hematologists with the fundamental knowledge required for understanding and managing these patients who become suddenly and seriously ill during pregnancy and in whom thrombocytopenia and microangiopathic hemolytic anemia are part of their presentation.

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.

Background Richter’s syndrome is the transformation of indolent lymphoma into an aggressive form, most commonly diffuse large B cell lymphoma. Most patients are known to have chronic lymphocytic leukemia. Richter’s syndrome arises in the lymph nodes or bone marrow and rarely presents with extra nodal involvement. Case presentation Our patient presented with Richter’s syndrome at an extremely uncommon site, i.e., testis. This is the fourth case reported in literature. Moreover, our case represents the longest follow-up reported so far with recurrence-free survival of more than 2 years now. Conclusion Testicular mass in a patient with a history of chronic lymphocytic leukemia should raise the clinical suspicion of Richter’s syndrome and accordingly the patient should be thoroughly investigated and treated. Treatment needs multidisciplinary team approach: urologist, pathologist and hematologist.

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.

A 25-year-old male (Case 1) was waiting for a bone marrow transplant for myelodysplastic syndrome. Due to acute appendicitis, he was advised to undergo gastroenterological surgery. After blood transfusion, he underwent an emergency laparoscopic appendectomy, as no blood cell recovery was expected. The postoperative course was uneventful, and he was discharged. A 71-year-old female (Case 2) developed acute appendicitis during chemotherapy for acute myeloid leukemia (AML). At the time of onset, since her myelosuppression was expected to improve in approximately 1 week, a conservative treatment was administered. However, due to the progression of AML, the expected blood cell recovery did not occur. Therefore, laparoscopic appendectomy was performed 25 days after onset. She was discharged without postoperative adverse events. In cases of acute appendicitis in patients with hematologic disease accompanied by pancytopenia, it is important to establish a careful treatment plan considering the possibility of recovery from myelosuppression and the need to control an intraperitoneal infection in conjunction with a hematologist. Laparoscopic surgery, which is minimally invasive, was an effective surgical procedure.

Multiple myeloma occupies a leading position among malignant blood diseases. There is clinical evidence that multiple myeloma can be complicated by secondary amyloidosis. The article presents a clinical case for the diagnosis of multiple myeloma complicated by secondary pulmonary amyloidosis. The participation of doctors of various specialties (pulmonologist, hematologist, pathologist), the use of high-tech diagnostic methods (histological examination of a biopsy of lung tissue, trepanobiopsy, immunohistochemical examination of histological preparations of lungs and bone marrow), made it possible to establish a clinical diagnosis.

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.

Hereditary angioedema (HAE) is a rare autosomal dominant disorder characterized by episodes of localized swelling, often of life-threatening severity. HAE due to C1 inhibitor (C1-INH) deficiency is common and is divided into types 1 and 2, but HAE with normal C1-INH is exceedingly rare. Herein, we describe the case of a patient with HAE with normal C1-INH undergoing orthognathic surgery. A 24-year-old woman came to our hospital with a diagnosed jaw deformity and commenced preoperative orthodontic treatment before scheduled orthognathic surgery. During the preoperative period, she experienced mild hoarseness. The hoarseness worsened, and computed tomography revealed mild laryngeal edema. Serum C1-INH, C3, C4, and CH50 levels were normal. Her younger sister had reportedly experienced a similar episode of lip edema previously. These findings supported a diagnosis of HAE with normal C1-INH. She underwent orthognathic surgery in close consultation with a hematologist and anesthesiologist at the age of 33 years. The surgery was completed without complications. She reported throat tightness 4 days after surgery, although her facial swelling was consistent with the procedure performed and was not remarkable. Concentrated C1-INH was administered, and the throat tightness slowly resolved within approximately 1 hour. Twenty-five days after surgery, she was discharged with reduced facial swelling. In the present case, orthognathic surgery was performed successfully in a patient with the exceedingly rare condition of HAE with normal C1-INH, in close consultation with a hematologist and an anesthesiologist. Postoperative throat tightness was successfully treated via the administration of concentrated C1-INH. Concentrated C1-INH administration can be considered in patients with HAE and normal C1-INH who experience angioedemic attack, keeping in mind that it may be slow to take effect.

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.

T cell’s journey begins from the bone marrow and ends in the peripheral tissues by either fighting the infections or residing in the secondary lymphoid organs as memory T cell. Thymus plays most important role in transforming a docile T cell precursor into a warrior. T cell is one of the most important cells in health and disease. Various diseases originate from qualitative or quantitative defects in T cells leading to various inherited and acquired diseases. This simple and short review is focused on the basic understanding and clinical hematologist’s perspective of T cells.

Was ist neu? CRS und ICANS als Nebenwirkung von CAR-T-Zellen Das Cytokine-Release-Syndrome (CRS) ist die häufigste Nebenwirkung einer CAR-T-Zell-Therapie und kann von leichtem Fieber bis zu einem Multiorganversagen führen. Pathophysiologisch kommt es beim CRS zu einem Zytokinsturm und trotz einer Therapie mit Tocilizumab sind refraktäre und tödliche Verläufe beschrieben. Die Symptome des Immune-Effector-Cell-associated-Neurotoxicity-Syndrome (ICANS) variieren von leichter Desorientiertheit bis zum lebensbedrohlichen Hirnödem. Die Pathophysiologie und Therapie des ICANS sind noch nicht ausreichend erforscht. Die Differenzialdiagnosen von CRS und ICANS sind komplex und umfassen neben Infektionen und Sepsis unter anderem auch eine Toxizität der vorhergehenden Therapie, ein Tumorlysesyndrom und nicht zuletzt einen Progress der Grunderkrankung. Ein klinischer oder laborchemischer Parameter zum sicheren Beweis oder Ausschluss eines CRS oder ICANS gibt es zum heutigen Zeitpunkt nicht. Intensivmedizinische Relevanz und potenzielle Entwicklungen der CAR-T-Zell-Therapie Erste Auswertungen von Real-world-Daten deuten auf eine höhere Rate an schweren Nebenwirkungen im Rahmen der CAR-T-Zell-Therapie als in den Zulassungsstudien hin. Für die Indikation r/r-DLBCL könnten schätzungsweise bis zu maximal 300 Patienten pro Jahr in Deutschland eine intensivmedizinische Betreuung im Rahmen der CAR-T-Zell-Therapie benötigen. Studien mit wesentlich häufigeren soliden Tumoren könnten die Patientenzahl drastisch erhöhen. Therapieziel bei CAR-T-Zell-Patienten und Entscheidungen bei Therapiezieländerung Aufgrund des neuen Therapiekonzepts kann ein Konflikt zwischen bislang palliativem Patientenkollektiv und nun möglicherweise langfristigen Remissionen entstehen. Eine frühzeitige Aufklärung über potenziell lebensbedrohliche Nebenwirkungen im Rahmen der Therapie und eine interdisziplinäre Besprechung der Therapieziele mit den Patienten ist entscheidend.

Objectives The objective of this study was to highlight the role of the clinical laboratory and the relevance of reporting the case immediately to the unit of hematology for the diagnosis and early administration of treatment in the presence of such an urgent hematologic disease as thrombotic thrombocytopenic purpura (TTP). Case presentation An elderly patient was referred to the emergency department of our hospital by his general practitioner for speech difficulty, facial asymmetry and weakness in the upper limb. Stroke code was activated. However, laboratory findings (anemia, thrombocytopenia, elevated creatinine, total bilirubin and LDH, negative direct Coombs test) and presence of schistocytes in the peripheral blood smear test were consistent with a completely different diagnosis: TTP thrombotic microangiopathy. Conclusions The first diagnostic approach of left hemispheric stroke was not confirmed in the laboratory, with findings of nonautoimmune hemolytic anemia, thrombocytopenia without apparent cause and presence of schistocytes. We should not forget that the clinical manifestations of this condition are widely variable and may include multiorganic dysfunction. Although confirmation of diagnosis is based on ADAMTS-13, its associated high mortalitiy requires immediate treatment on mere suspicion.

The acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. However, unlike the inherited disease, AvWS occurs in persons with no personal and family history of bleeding and is often associated with a variety of underlying diseases, most frequently lymphoproliferative, myeloproliferative and cardiovascular disorders. After the presentation of a typical case, in this narrative review we discuss the more recent data on the pathophysiology, clinical, laboratory and therapeutic aspects of this acquired bleeding syndrome. We chose to focus particularly on those aspects of greater interest for the hematologist.

BACKGROUND Factor X deficiency (known as; Stuart-Prower factor deficiency or F10 deficiency) is a rare inherited bleeding disorder. It affects one per 1 million individuals worldwide. Patients with inherited bleeding disorder reported difficulty in accessing primary dental care either due to their medical diseases or their related barriers. AIM This article aims to identify barriers to oral health as perceived by the patient with factor X deficiency who had been refused treatment by three dentists. A further aim is to provide dentists with the knowledge required to manage patients diagnosed with factor X deficiency. METHODS AND CASE REPORT A 30-year-old male with the inherited, severe factor X deficiency (

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.

Chimeric antigen receptors (CARs) are genetically engineered receptors that provide specific properties to an immune effector cell and these receptors gain the specificity of a monoclonal antibody targeted against specific tumor cells. T cells with engineered CARs acquire potent immunological properties and redirect the immune system in order to eliminate malignant cells. First-engineered T cells with chimeric molecule (CAR-T cells) were developed in 1989–1993 by Israeli immunologists Zelig Eshhar and Gideon Gross. The first clinical application of CAR-T cells was done in the University of Pennsylvania and Children’s Hospital in Philadelphia by the immunologist Carl June and hematologist David Porter to patients with chronic lymphocytic leukemia in 2011 and together with the pediatrician Stephan Grupp to patients with acute lymphoblastic leukemia (ALL) in 2012. The US Food and Drug Administration Agency (FDA) in 2017 and the European Medicines Agency (EMA) in 2018 have licensed two products of CAR-T cells: tisagenlecleucel for the use in children and young adults up to 25 years of age with B-cell ALL who do not respond to treatment or have relapsed two or more times and tisagenlecleucel and axicabtagene ciloleucel for the use in adult patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL). Current progress in CAR technology includes the use in other hematological malignancies, solid tumors, the use of dual CAR-T cells and chimeric antigen receptor natural killer cells (CAR-NK cells).

Aim Orodental manifestations are commonly presented in Wiskott Aldrich Syndrome (WAS). The purpose of this paper is to report a case of dental management of a 5-year-old male child with WAS before Hematopoietic Stem Cell Transplantation (HSCT). Such patients are more prone to infection due to pretransplantation chemotherapy and posttransplantation immunosuppression; thus, it becomes imperative to eliminate all potential sources of infection before transplantation. Materials and methods Fluctuating blood parameters before the dental procedure was an important challenge in rendering dental treatment. Dental procedures were carried out under general anesthesia by maintaining the hematological parameters with blood and platelet transfusion. The conventional dental treatment may not be applicable in such patients as failure of dental treatment can cause the failure of HSCT, and it has to be modified based on the clinical acumen and recommendations. Conclusions This case report focuses on the measures to be taken before, during, and after the dental procedure to ensure the success of the dental therapy and prevent failure of HSCT due to residual dental foci of infection. A multidisciplinary approach involving a pedodontist, a pediatrician, and a hematologist can improve the quality of life of such patients.

The purpose of this study was to compare clinical decision-making in iron overload patients using FerriScan and an R2*-based approach. One-hundred and six patients were imaged at two consecutive timepoints (454 ± 158 days) on a 1.5-T Siemens MAGNETOM Avanto Fit scanner. For both timepoints, patients underwent the standard FerriScan MRI protocol. During the second exam, each patient additionally underwent R2*-MRI mapping. For each patient, a retrospective (simulated) decision was made to increase, decrease, or maintain chelator levels. Two different decision models were considered: The fixed threshold model assumed that chelator adjustments are based strictly on fixed liver iron concentration (LIC) thresholds. Decisions made with this model depend only on the most recent LIC value and do not require any clinician input. The second model utilized decisions made by two hematologists retrospectively based on trends between two consecutive LIC values. Agreement (κA) between hematologists (i.e., interobserver variability) was compared with the agreement (κB) between a single hematologist using the two different LIC techniques. Good agreement between R2*- and FerriScan-derived decisions was achieved for the fixed threshold model. True positive/negative rates were greater than 80%, and false positive/negative rates were less than 10%. ROC analysis yielded areas under the curve greater than 0.95. In the second model, the agreement in clinical decision-making for the two scenarios (κA vs. κB) was equal at the 95% confidence level. Switching to R2*-based LIC estimation from FerriScan has the same level of agreement in patient management decisions as does switching from one hematologist to another. • Good agreement between R2*- and FerriScan-derived decisions in liver iron overload patient management • Switching to R2*-based LIC estimation from FerriScan has the same level of agreement in patient management decisions as does switching from one hematologist to another.

Women with inherited bleeding disorders, including carriers of hemophilia A and B, or with von Willebrand disease, have an increased risk of bleeding during pregnancy and delivery. The unborn child may also be affected by the bleeding disorder for which specific measures have to be considered. This requires a multidisciplinary approach, with a team that includes a hematologist, a pediatric hematologist, a clinical geneticist, an obstetrician-perinatologist, and an anesthesiologist. An optimal approach includes prepregnancy genetic counseling, prenatal diagnostic procedures, and a treatment plan for delivery for both the mother and child. Recent retrospective studies show that even if strict guidelines are followed, these women are still at risk of postpartum bleeding. This occurs even if coagulation factor levels are normalized, either due to the pregnancy-induced rise of factor levels or by infusion of coagulation factor concentrates at the time of delivery. In this article, we describe our current diagnostic and clinical management of pregnancy and delivery in women with inherited bleeding disorders. We also briefly discuss possible interventions to improve the outcome of current strategies by increasing target factor levels during and after delivery.

Introduction. Accessory spleen represents ectopic spleen tissue separated from the body of the spleen, with the percentage share of 10?15% in a population. Case outline. We present a female patient in which immune thrombocytopenic purpura was diagnosed 12 years previously and, after a failed initial treatment, it was decided by a hematologist to perform a laparoscopic splenectomy. The mentioned operation was carried out in a safe and efficient manner wherein the accessory spleen was detected and removed intraoperatively. The operative and postoperative course passed without any complications. The definitive histopathological findings confirmed previously set hematological diagnosis. Conclusion. The laparoscopic approach is a superior modality in terms of diagnostic and therapeutic procedures when it comes to surgical removal of the accessory spleen. Taking into consideration the advantages of this approach presented and proven in literature, even in the case of diagnostically or intraoperatively overlooked accessory spleen or de novo discovered after the operation, there should be no dilemma which surgical approach should be applied.

A 59-year-old man with Hodgkin's lymphoma was referred by a hematologist for consultation for cutaneous issues. Physical examination revealed generalized scaling and erythematous scaly patches located in the groin, abdomen, and arms. The biopsy was compatible with mycosis fungoides (MF). At his next medical visit, painful nodules with erythematous halo and scabby surface were noted, and a subsequent biopsy was compatible with lymphomatoid papulosis (LyP). Mycosis fungoides, the most common primary cutaneous T-cell lymphoma, is usually defined in its classic form as a CD4+ non-Hodgkin lymphoma; LyP corresponds to a CD30+ lymphoproliferative disorder; and Hodgkin's lymphoma (HL) constitutes a lymphoid neoplasia characterized by the presence of Reed–Sternberg cells and its variants. Although these entities have been defined independently, evidence suggests the possibility of a common monoclonal origin. To our knowledge, this is the first case of MF, LyP, and HL in a single patient.

Background: Sickle-cell disease (SCD) causes significant morbidity, premature mortality, and high disease burden, resulting in frequent health care use. Comanagement may improve utilization and patient adherence with treatments such as Hydroxyurea. The purpose of this study was to describe acute-care utilization in Medicaid-enrolled patients with SCD, patient factors associated with comanagement, and adherence to Hydroxyurea. Methods: Data from 2790 patients diagnosed with SCD, age 1 to 65+ years, enrolled at least 1 month in North Carolina Medicaid between March 2016 and February 2017, were analyzed. Outpatient visits were categorized as primary care, hematologist, and nonhematologist specialist. Nurse practitioners or physician assistants with unidentified specialty type or family practice were categorized separately. Comanagement was defined as a minimum of 1 primary care and 1 hematologist visit/patient during the study period. Results: There were notable age-related differences in utilization of health care services. Only 34.82% of the sample was comanaged. Comanagement was higher in the 1-to-9-year-old (44.88%) and 10-to-17-year-old groups (39.22%) versus the 31-to-45-year-old (30.26%) and 65+-year-old (18.75%) age groups. Age had the greatest influence (AUC = 0.599) on whether or not a patient was comanaged. Only a third of the sample (32.24%) had at least 1 Hydroxyurea (HU) prescription. Age was the most predictive factor of good HUadherence (AUC = 0.6503). Prediction by comanagement was minimal with an AUC = 0.5615. Conclusion: Comanagement was a factor in predicting HUadherence, but further studies are needed to identify the frequency and components of comanagement needed to increase adherence and reduce acute care utilization.

Myeloproliferative neoplasms (MPN) are traditionally regarded as a disease of older adults, though a not negligible fraction of cases occurs at a younger age, including women of childbearing potential. MPN in younger patients, indeed, offer several challenges for the clinical hematologist, that goes from difficulties in reaching a timely and accurate diagnosis to a peculiar thrombotic risk, with a relatively high incidence of thromboses in unusual sites (as the splanchnic veins or the cerebral ones). Moreover, the issue of pregnancy is recently gaining more attention as maternal age is rising and molecular screening are widely implemented, leading to a better recognition of these cases, both before and during pregnancy. In the present work we aim at discussing four clinical topic that we identified as areas of uncertainty or true unmet medical needs in the management of younger patients with MPN, with a particular focus on the topic of pregnancy. For each of these topics, we critically reviewed the available evidence that support treatment decisions, though acknowledging that recommendations in this field are mostly based on expert opinion or derived from guidelines of other clinical conditions that share with MPN a high vascular risk, as antiphospholipid syndrome. Taking into consideration both the lack of evidence-based data and the clinical heterogeneity of MPN, we support an individualized strategy of counseling and management for both young patients and for expectant mother with MPN.

Coronavirus disease 19 (COVID‐19) is considered a multisystemic disease. Several studies have reported persistent symptoms or late‐onset complications after acute COVID‐19, including post‐COVID‐19 hematological disorders. COVID‐19‐induced coagulopathy, an immunothrombotic state, has been linked to thromboembolic and hemorrhagic events. Late‐onset thrombocytopenia related to immune system dysregulation has also been reported as a rare manifestation post COVID‐19. Close monitoring of laboratory dynamics is considered essential to identify timely abnormal values that need further investigation, providing supportive care whenever indicated. The role of hematologists is essential in terms of the multidisciplinary approach of long COVID‐19. This review summarizes all the available evidence on post‐acute COVID‐19 hematological complications.

Despite the emergence of more effective targeted therapies, cancer treatment remains a complex process requiring a holistic patient-centered approach, beyond the direct medical care offered by the treating hematologist/oncologist. This entails empowering patients with knowledge about prescribed regimens and their risks and side effects, within a multifaceted treatment team involving hematologists/oncologists, advanced practice providers, nurses, nutritionists, and pharmacists, among others. Additionally, a multitude of resources are generally available including financial, religious, and spiritual support to help patients in the treatment journey. This chapter describes resources generally available to cancer patients, as well as an array of supporting services in a cancer center to address the patient needs.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition and furthermore human immunodeficiency virus (HIV)-associated HLH is rarely reported in the literature. The most frequent presentation of secondary HLH is in association with infections, malignancies or drugs. In HIV-positive patients, the viral infection itself or the antiretroviral therapy (ART) could trigger HLH. Case presentation: A 14-year-old boy was admitted for persistent diarrhea, severe weight loss and chest burns. Laboratory tests revealed important neutropenia, which led to HIV infection diagnosis. ART in combination was started associated with granulocyte-colony stimulating factor. Viral copies declined after six weeks of treatment, but the cluster of differentiation 4 (CD4) T-lymphocytes and neutrophil counts remained very low. Infections and malignancies were ruled out. The bone marrow aspirate revealed hemophagocytosis which in association with fever, bicytopenia, hypofibrinogenemia and hypertriglyceridemia established HLH diagnosis. Cortisone therapy and intravenous immunoglobulins were added. Due to lack of response, HLH-2004 protocol was initiated in collaboration with pediatric hematologist. In the first six months of treatment, CD4 T-lymphocytes and neutrophil count remained low and then they showed significant increase simultaneously. During treatment, the patient developed spontaneous severe back pain. Magnetic resonance imaging showed vertebral compression, osteomalacia and a thoracic vertebral fracture. Conclusions: Having ruled out the usual associated infections and malignancies, this is a case of HIV-associated with HLH. In this case, only the ART and immunomodulatory therapy were not enough and failed to work. Systemic immunosuppression also worked only after a prolonged course which led to an unfortunate complication: a vertebral fracture.

A 16-year-old male Type A hemophiliac patient has been reported with complaints of defective vision in the right eye (OD), following ocular trauma 9 months back for which he took treatment outside. On examination, he had blood staining of cornea and cataract in his right eye. His vision was perception of light + and projection of rays intact. After hematologist opinion, under peribulbar anesthesia, he underwent penetrating keratoplasty with pupillary membranectomy and cataractous lens removal with posterior chamber intraocular lens implantation under the cover of Factor VIII (antihemophilic factor). On day 21, the best-corrected visual acuity in OD was 6/6P. This case report emphasizes the fact that hemophiliac patients can be operated safely with appropriate hematological support.

In this article, 2 clinical cases of patients with hemostatic problems are considered. 1 case during pregnancy, with confirmed APS and received therapy. The pathology of the hemostasis system of congenital or acquired genesis against the background of pregnancy can lead to an unfavorable outcome in the form of non-developing pregnancy and miscarriage, and maternal mortality. 2 case with autoimmune nature of APS diagnosed at late stages. It is concluded that excessive examination for non-informative indicators can lead to an incorrect interpretation of blood parameters and, as a consequence, to polypharmacy and a protracted course of the disease with the development of life-threatening complications.

CADTH recommends that Vyxeos should be reimbursed by public drug plans for the treatment of adults with newly diagnosed therapy-related acute myeloid leukemia (t-AML) or AML with myelodysplasia-related changes (AML-MRC) if certain conditions are met. Vyxeos should only be reimbursed if the induction cycles are administered in an inpatient setting and supervised by a hematologist with expertise in managing patients with acute leukemia and if the cost of Vyxeos is reduced. Vyxeos should only be covered to treat adult patients with newly diagnosed t-AML or AML-MRC who were deemed fit for intensive chemotherapy by the treating physician.

Coronavirus disease 2019 (COVID-19) is impacting our daily practice. Hematologist and oncologist around the globe are concerned and many physicians are hesitating to start/continue antineoplastic treatment in patients with cancer to protect them from severe complications in case of infection with the new novel corona virus. Therefore, we feel there is a high demand for scoring system to guide and help physicians dealing with patients with malignant diseases to take the correct decision. We have designed a scoring system to evaluate patients with cancer requiring anti-cancer treatment and we have started to implement it at our hospital. Therefore, we would like to share this scoring system with the community.

Despite huge progress in the fields of newborn screening, encapsulated bacterial infection prophylaxis, immunization, and supportive care in general, people suffering from sickle cell anemia still continue to have a shorter life expectancy and a poorer quality of life due to painful vaso-occlusive events and strokes during childhood, and later, cardiac, pulmonary, and renal injuries, including in Western and high-income countries. From the 2000s, allogeneic stem cell transplantation for severe sickle cell disease from a sibling donor provided the best results-overall as well as disease-free survival-never obtained for any other disease. Nevertheless, this only curative option is proposed to few patient numbers, including in Western countries with high-level medical equipment development, with discrepancies between (i) patients and family, (ii) physicians and care centers dedicated to sickle cell disease, and (iii) hematopoietic stem cell transplant teams. Due to these discrepancies and in order to provide the same quality of discussion and treatment choice for every sickle cell disease patient, we developed a National French multidisciplinary pluri-annual meeting dedicated to sickle cell disease patients and transplantation. We report here our experience of such a meeting.