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IgA nephropathy (IgAN) is the most frequent type of primary glomerulonephritis since the first type was described more than four decades ago. It is the prevalent cause of primary glomerular disease that causes end-stage renal disease. In most patients with IgAN, hematuria is the most common reported symptom, particularly in those with a preceding upper respiratory tract infection. Although the pathogenesis of IgAN is usually multifactorial, autoimmune complex formation and inflammatory processes are the most widely recognized pathogenic mechanisms. Multiple approaches have been trialed as a therapy for IgAN, including tonsillectomy, steroids, other immune-suppressive therapy in different regimens, and kidney transplantation.PubMed, Google, Google Scholar, Scopus, and EMBASE were searched by the authors using different texts, keywords, and phrases. A non-systemic clinical review is intended to review the available data and clinical updates about the possible mechanism(s) of IgAN pathogenesis and treatments.IgAN has a heterogeneous pattern worldwide, making it difficult to understand its pathogenesis and treatment. Proteinuria is the best guide to follow up on the IgAN progression and treatment response. Steroids are the cornerstone of IgAN therapy; however, other immune-suppressive and immune-modulative agents are used with a variable response rate. Kidney transplantation is highly advisable for IgAN patients, although the recurrence rate is high. Finally, IgAN management requires collaborative work between patients and their treating physicians for safe long-term outcomes.

A novel rapid spreading and changing virus called SARS-CoV-2 appeared in Wuhan city in December 2019. It was announced by the World Health Organization (WHO) as a pandemic disease in March 2020. It commonly presents with respiratory symptoms; however, it may be asymptomatic. Electrolyte abnormalities are not uncommon features of SARS-CoV-2 infection. Hyponatremia is one of these electrolyte disturbances among SARS-CoV-2 patients, and it may produce symptoms such as weakness and seizure as the initial presenting symptoms. The underlying mechanism(s) of hyponatremia due to SARS-CoV-2 infection is (are) not established. The aim of this review is to evaluate the possible mechanism of hyponatremia in patients with COVID-19. Understanding and categorizing the hyponatremia in these patients will lead to better treatment and correction of the hyponatremia. A review of the literature between December 2019 and March 2022 was conducted searching for the possible reported mechanism(s) of hyponatremia in SARS-CoV-2. Although SIADH is the commonly reported cause of hyponatremia in SARS-CoV-2 infection, other causes such as diarrhea, vomiting, and kidney salt loss must be considered before SIADH.

Introduction and importance Coronavirus disease 2019 (COVID-19) is a recently discovered disease that has yet to be thoroughly described. It is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel virus that can be transmitted easily from human to human, mainly by the respiratory route. The disease often presents with non-specific symptoms such as fever, headache, and fatigue, accompanied by respiratory symptoms (e.g., cough and dyspnea) and other systemic involvement. Currently, vaccination is the primary strategy to prevent transmission and reduce disease severity. However, vaccines have side effects, and the consequences of vaccination in different diseases are not well established. Moreover, the impact of SARS-CoV-2 vaccination during pregnancy is another not well-known area. Case presentation We present a young lady known to have ITP, which was controlled for years, presented with relapse after taking the SARS-CoV-2 vaccine during pregnancy. Clinical discussion The patient had a relapse of ITP after the introduction of the first dose of the COVID-19 vaccine, which worsened further after the second dose. This suggests that patients with ITP who develop flare post-SARS-CoV-2 vaccine should have their second dose delayed, particularly if pregnant. Conclusion To avoid further deterioration in platelet count, and avoid confusion due to the presence of different causes of thrombocytopenia and avoid complications related to thrombocytopenia during pregnancy which can affect the mode of delivery. The case is reported in line with the scare 2020 criteria Agha RA, Franchi T, Sohrabi C, Mathew G, for the SCARE Group. The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines, International Journal of Surgery 2020; 84:226–230.

An 11-month-old full-term female infant was referred to the hematology clinic due to marked anemia and neutropenia. She was almost exclusively breastfed and rejecting all trials for supplementary food including artificial formulas. Bone marrow aspirate revealed cytoplasmic vacuolization in precursors of the myeloid and erythroid series with significant dysgranulopoiesis and dyserythropoiesis and ringed sideroblasts. Flow cytometry analysis revealed increased hematogones with aberrant loss/downregulation of CD33 on granulocytes and monocytes (sign of dysmyelopoiesis). Laboratory investigation revealed low serum copper and ceruloplasmin. Administration of a multivitamin including a high concentration of copper for only 1 week improved her hemoglobin and absolute neutrophil count up to 1.9 × 103/µL, then dropped to 0.3 103/µL after she stopped taking the copper multivitamin. Her blood counts improved till total normalization and up to the time this report is issued. The probable role of unrecognized copper deficiency in causing anemia in infants more than 6 months of age is discussed, and the importance of serum copper examination in refractory anemia and neutropenia is emphasized. This case shows that copper deficiency should be an integral part of the differential diagnosis of refractory anemia including sideroblastic anemia and dysplasia. To the best of our knowledge, no such case has previously been described in the literature.

Although eslicarbazepine is an anti‐seizure medication, it may result in seizure worsening if its use is complicated by severe hyponatremia, even long time after commencing this medication. The treatment is a replacement for another ASM.

Objective Following the launch of the TKI's (tyrosine kinase inhibitors) for the treatment of CML, establishing its significant control over the disease, other dimensions have emerged in regard to the safety of treatment, particularly the effect on Male fertility and fatherhood. This study was conducted to review the real-life data on the effect of TKI on the fertility of male patients in the National Center of cancer care and research (NCCCR) in Qatar. Case report Inclusion Criteria: Male patient diagnosed with CML, in Chronic or accelerated phase; 18 years of age or older and actively receiving tyrosine kinase inhibitors including (Imatinib, dasatinib,nilotinib) with the following: -Patients with no known issues with regards to fertility, (fertility is intact) Patients who developed fertility issues after the diagnosis of CML and starting TKI's. has been evaluated by an andrologist, and his evaluation concluded its TKI related. Methodology A single-center study conducted a mixed-design study by phone interviews with CML male patients in the Chronic or accelerated phase, being followed up in NCCCR (national center for cancer care and research), evaluating the effect of Imatinib, Dasatinib, nilotinib, on their fertility whether they are taking it as first, a second, or third line of treatment. Results 150 patients were interviewed to be included in the study, 22 patients had concerns related to medications safety and possible transmission of the disease, 33 patients had their families completed by the time of diagnosis. 26 patients have met the inclusion criteria, offspring's total number was 43, 97.6% were full-term, had a normal delivery, and normal average weight at delivery. No stillbirths, fetal demise, or congenital anomaly were reported. All offspring had normal development and growth. Conclusion Around 98% of male CML patients taking imatinib, Dasatinib, Nilotinib had their offspring born normally with no delivery complications noted, all had no congenital anomaly had normal growth and development, and no CML-related cancers were diagnosed. Further studies with a larger sample size are required to shed light on the TKI outcome on fatherhood in CML patients.

Philadelphia negative myeloproliferative neoplasms (MPNs) are classically characterized by excess production of terminal myeloid cells in the peripheral blood. They include polycythemia vera, essential thrombocythemia, and primary myelofibrosis. Among this group, primary myelofibrosis is the least common and usually carries the worst prognosis. Bone involvement in primary myelofibrosis has many forms; it affects bone marrow leading to bone marrow fibrosis, it can cause periostitis, in addition to bone and joint pain. A common radiologic finding in primary myelofibrosis is the presence of osteosclerotic lesions. However, the presence of osteolytic lesions in bone imaging was described in few reports. In this review, we searched English literature using the PRISMA guidelines looking for patients with Primary myelofibrosis who had osteolytic bone lesions to assess the impact of such findings on the disease and its effect on prognosis. We found the vast majority of lesions were painful affecting most commonly the vertebral column, pelvis, and ribs, and were detected in patients above 50 years of age with no gender preference, unfortunately they represented advanced disease stages, resulting in inadequate treatment response and poor outcome.

Thalassemia is a hematologic disorder caused by genetic mutation resulting in impaired hemoglobin chain production. Patients with thalassemia commonly experience complications such as anemia, blood transfusion-related issues, hepatic or cardiac involvement, and psychosocial impacts. Rarely, priapism has been associated with thalassemia as an initial presentation or subsequently occurring at any time in the disease course. Our literature review summarizes the reported cases of thalassemia-associated priapism and delves into underlying mechanisms of its pathophysiology and appropriate management.

Idiopathic thrombocytopenic purpura (ITP) is an immune disorder in which antibodies attack platelets, leading to platelet destruction and increased bleeding risk. Standard treatment is to maintain a platelet count sufficient to mitigate the bleeding risk. First-line therapies include steroids and IV immunoglobulins, and second-line therapy includes thrombopoietin receptor agonists like eltrombopag in combination with other medications (e.g., rituximab) to reduce immune attack. Eltrombopag is a nonpeptide oral thrombopoietin (TPO)-receptor agonist that increases platelet counts by binding to and activating the human TPO receptor. While using eltrombopag, the target platelet count range is usually between 50,000/mm³ and 200,000/mm³, so the dose should be adjusted accordingly. However, this dose adjustment is based on platelet count increments in response to eltrombopag administration. Adjusting the dose when the platelet count is elevated due to a different factor can be challenging. Data are not yet available on whether stopping the treatment or reducing the dose will harm the patient or result in an acute drop in platelet count and increased bleeding. We present the case of a 60-year-old woman with ITP on a stable eltrombopag regimen who completed an eltrombopag-free period after developing infection-induced thrombocytosis.

Priapism is defined as a persistent penile erection lasting more than 4 h. We searched the literature for reviews, case reports, and series for patients with lymphoproliferative disorders who developed priapism. The search involved all the lymphoproliferative disorders included in the revised 2016 World Health Organization classification of lymphoid neoplasms including chronic lymphocytic leukemia, multiple myeloma, Waldenstrom macroglobulinemia, and lymphomas. A total of 16 articles were found. The search included cases up to 4th January 2021. Priapism was seen most commonly as the first manifestation of lymphoproliferative disorders, rarely seen after treatment or after diagnosis.

Immune thrombocytopenic purpura (ITP) is a blood disorder in which antibodies coating platelets cause platelets destruction in the spleen with resultant low platelets count and an increased tendency for bleeding. Coronavirus disease 2019 (COVID-19) is an illness caused by SARS-COV2; it was first identified in December/2019; though it mainly affects the respiratory system, multisystemic complications are identified. Several ITP cases post mRNA SARS-CoV-2 vaccines were reported, and different pathophysiology theories about the underlying pathophysiology were discussed, but only a few ITP relapse cases have been reported so far. We present a 28-year-old Asian male, a known patient of ITP and in partial remission for eighteen months, who presented to the emergency department with ITP relapse (platelets count of 1 × 10^3 /µL), four days after receiving the second dose of Pfizer SARS-CoV-2 vaccine, which required treatment with intravenous immunoglobulins and dexamethasone. We further discuss the preferred approach in ITP patients who are willing to receive the COVID-19 vaccine.

Gastrointestinal manifestations of leukemias have been well recognized. Typically, acute leukemias cause typhlitis or appendicitis more commonly than chronic leukemias. Our case points to appendicitis as possible manifestation of chronic myelogenous leukemia.

Patient: Male, 49-year-old Final Diagnosis: Chronic lymphocytic leukemia • COVID-19 Symptoms: Feve • shortness of breath Medication: — Clinical Procedure: — Specialty: Hematology • Infectious Diseases Objective: Unusual clinical course Background: COVID-19 is a newly emerging disease that is not yet fully understood. It is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel virus that is easily transmitted from human to human through the respiratory route. Usually, it presents with fever, headache, fatigue accompanied by respiratory symptoms like cough and dyspnea, and other systemic involvements. Chronic lymphocytic leukemia (CLL) is a common lymphoproliferative neoplasm characterized by absolute lymphocytosis and demonstration of clonality unlike other causes of lymphocytosis. Patients with CLL are considered immunocompromised because of impaired humoral immunity (mainly) and cellular immunity. Therefore, they are vulnerable to various infections including COVID-19. Little is known about the COVID-19 infection when it unmasks CLL. Case Report: A 49-year-old man with no significant previous illnesses, and an unremarkable family history, presented with a moderate COVID-19 infection. He initially presented to the emergency department with fever and mild shortness of breath. A complete blood count showed a high white blood cell count with absolute lymphocytosis. Flow cytometry revealed the clonality of the lymphocytes confirming the diagnosis of CLL. Despite having CLL, he developed a moderate COVID-19 infection and recovered in a few days. To the best of our knowledge, this is the first report of CLL, which presented with a COVID-19 infection as the initial presentation. Conclusions: Lymphocytosis is an unexpected finding in patients diagnosed with COVID-19 infection and the elevated lymphocytes may be indicative of other conditions. Secondary causes of lymphocytosis like malignancy or other infections should be considered in these cases.

Background: Electronic devices for measuring blood pressure (BP) need to go through independent clinical validation as recommended by different authorities, both in general and specific populations. The aim of this study was to assess the validity of the Omron RS6 (HEM-6221-E) wrist oscillometric devices in obese Sudanese patients. Methods: Of 90 obese individuals invited for recruitment, 33 were included in the study, and had their BP at the level of the wrist measured using Omron RS6 and standard mercury sphygmomanometer. Two observations were made and the mean was taken. BP differences between the two methods for the 33 participants were classified into three categories (≤5, ≤10, and ≤15 mmHg), according to the European Society of Hypertension-International Protocol revision 2010 (ESH-IP2) criteria. This was then used to assess the validity of the tested Omron RS6 device. Results: Participants had a mean age of 56.97 years (standard deviation (SD), 8.75; range, 36-79). Average systolic blood pressure (SBP) was 146.21 mmHg (SD, 23.07; range, 107-182), and average diastolic blood pressure (DBP) was 93.82 mmHg (SD, 16.06; range, 67-128). There was a good agreement between the two observations using the OMRON RS6 and the standard sphygmomanometer: −4 to + 3 mmHg for SBP and −4 to +4 mmHg for DBP, with the mean difference of 1.73±1.11 mmHg for SBP and 1.49±1.02 mmHg for DBP. Conclusion: Thus, the Omron RS6 (HEM-6221-E) is a valid and suitable measure of BP according to ESH-IP2.

Patient: Male, 24-year-old Final Diagnosis: Acute kidney injury • coagulopathy • liver failure • SARS-CoV-2 Symptoms: Cough • fever Medication: — Clinical Procedure: — Specialty: Gastroenterology and Hepatology • Infectious Diseases Objective: Unusual clinical course Background: Coronavirus disease 2019 (COVID-19) is a newly emerging disease that is still not fully characterized. It is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel virus that can be transmitted easily from human to human mainly by the respiratory route. Currently, there is no specific treatment for COVID-19 or a vaccine for prevention. The disease has various degrees of severity. It often presents with non-specific symptoms such as fever, headache, and fatigue, accompanied by respiratory symptoms (e.g., cough and dyspnea) and other systemic involvement. Severe disease is associated with hemophagocytic syndrome and cytokine storm due to altered immune response. Patients with severe disease are more likely to have increased liver enzymes. The disease can affect the liver through various mechanisms. Case Report: We report an unusual case of SARS-CoV-2 infection in a 24-year-old man with no previous medical illness, who presented with mild respiratory involvement. He had no serious lung injury during the disease course. However, he experienced acute fulminant hepatitis B infection and cytokine release syndrome that led to multiorgan failure and death. Conclusions: It is uncommon for SARS-CoV-2 infection with mild respiratory symptoms to result in severe systemic disease and organ failure. We report an unusual case of acute hepatitis B infection with concomitant SARS-CoV-2 leading to fulminant hepatitis, multiorgan failure, and death.

Background: Priapism is defined as a penile erection that persists four or more hours and is unrelated to sexual stimulation. Priapism resulting from hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi and hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms. In patients with leukemia, 50% of cases of priapism are due to Chronic Myeloid Leukemia (CML). We present a systematic review of priapism in CML. Acquisition of evidence: An extensive literature research was carried out in PubMed, Google Scholar, SCOPUS, and Science Citation Index databases. The search included cases up to 4th August 2020. Synthesis of evidence: A total of 68 articles were found and included in our review, including 3 reviews from three different centers. We found 68 articles (102 patients; figure 1) and several case reports on priapism in CML. Priapism was noticed in some patients at the first presentation of CML. However, it was infrequently reported during the start of treatment, following the stop of medication and post-splenectomy. The mean age at presentation was 27.4 years, and the mean time from onset of priapism to the time to get medical attention (presentation) was 78.2 hours. The mean white blood cell count associated with priapism was 321.29x109/L, and the mean platelet count was 569 x10 9/L. The chronic phase of CML was the most common phase where priapism occurred. Most patients were Asian (>50%). Nearly a quarter of patients (27.4%) developed permanent erectile dysfunction. Figure 1. The PRISMA flow diagram detailing the cases of chronic myeloid leukemia (CML) presenting priapism. Conclusions: Priapism is a urological emergency requiring urgent multidisciplinary management to prevent erectile dysfunction. Because of the relatively rare occurrence of priapism in CML patients, there is no standard treatment protocol. (www.actabiomedica.it)

Sickle cell anemia (SCA) is a hereditary hemoglobin (Hb) disorder associated with a very specific molecular lesion, which is the exchange of glutamic acid for valine in the sixth residue of the Hb beta chain, originating the S Hb. It is characterized by intermittent episodes of vascular occlusion and end-organ damage. Progressive organ damage may affect any organ with brain, eyes, pulmonary, hepatobiliary, spleen, genitourinary, and the musculoskeletal systems being the most commonly involved and reported. Other complications of the disease that have not been well described or studied include cranio-orbital syndromes, oropharyngeal syndromes, periodontal disease, and otologic syndromes. Considering the vaso-occlusive nature of sickle cell disease (SCD), the potential for auditory damage is not unexpected. However, the incidence of subjective hearing impairment among SCA is very low and and little is known about the relationship between SCA and hearing loss. Here we report a 43-year-old female with SCA who presented with sudden bilateral hearing loss and generalized body ache and admitted as a case of sensorineural deafness with vascular crisis; she received IV fluid and analgesia and improved after five days from the therapy.

Introduction Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by overproduction of mature granulocytes. Up to half of the patients are asymptomatic and diagnosed during routine blood investigations; others present with fatigue and non-specific symptoms. Many patients develop gastrointestinal manifestations such as abdominal pain, bloody diarrhea, and pancreatitis during the disease course. Some presentations are related to leukemia itself, while others may be related to CML treatment. Methods We searched the English literature (PubMed, SCOPUS, and Google Scholar) for studies, reviews, case series, and case reports of patients with CML who developed any gastrointestinal manifestations involving the gastrointestinal tract from the esophagus down to the rectum Inclusion criteria comprised of patients above 18 years of age, with CML and gastrointestinal features. Pregnant women and bone marrow transplant recipients were excluded. Search terms included chronic myeloid leukemia, chronic myelogenous leukemia, with esophagitis, pancreatitis, duodenitis, gastritis, Crohn's disease, ulcerative colitis, inflammatory bowel disease, hepatocellular carcinoma, cholangiocarcinoma, colon cancer, malignancy, hepatitis, primary biliary cholangitis, primary biliary cirrhosis, primary sclerosing cholangitis, and perforation. Results A total of 129 patients were included. Patient characteristics are shown in table 1. Among the gastrointestinal manifestations, the most common treatment-related complications were drug-related hepatitis followed by reactivation of viral hepatitis B, pancreatitis, and typhlitis. Hepatitis in CML was reported with different TKIs but more commonly with imatinib. Reactivation of viral hepatitis B was common, while hepatitis C reactivation was rarely reported. Pancreatitis was associated mostly with nilotinib. Colitis is seen mainly with dasatinib. Inflammatory bowel diseases, liver diseases such as primary biliary cholangitis (PBC), were variable; some occurred after CML diagnosis while others preceded the diagnosis. Malignancies like pancreatic adenocarcinoma and hepatocellular carcinoma occurred after CML. Discussion Gastrointestinal features in patients with chronic myeloid leukemia can be the first presenting featuring of leukemia itself, arising during the course of CML or as a complication of the treatment. Interestingly, most of these presentations have been reported in patients with CML. These include inflammatory conditions such as pancreatitis and esophagitis, reactivation of viral hepatitis to the neoplastic process, and malignancy. In patients with CML, malignant tumors in the gastrointestinal tract can be caused by leukemic infiltration. Moreover, like other myeloproliferative neoplasms, CML confers a risk of developing a second non-hematological malignancy, including colonic neoplasms. Gastrointestinal complications can pose drastic impacts throughout the disease course; they may result in a change in the treatment, affect the prognosis, and may also be fatal, as in severe enterocolitis or fulminant liver failure. The treatment goal in patients with CML has changed significantly over the last decades. The current treatment goal is to achieve normal survival and good quality of life without the need for lifelong treatment. The improvement in CML treatment and prognosis is largely attributed to the introduction of tyrosine kinase inhibitors. However, most gastrointestinal features associated with treatment are related to tyrosine kinase. The exact pathogenesis of TKI injury is unclear but likely attributed to immune-related mechanisms. Imatinib is the first-line therapy for CML and is the most widely used TKI; however, not all the gastrointestinal features are associated with imatinib as expected. Various gastrointestinal features are prominent with other TKIs as well. Appropriately identifying which TKI is the likely trigger will help in avoiding highly suspected gastrointestinal complications or guide in switching to a safer TKI, thereby achieving treatment goals. Conclusion Patients with chronic myeloid leukemia can have a different gastrointestinal presentation which can alter their disease course. Such complications must be managed appropriately in order to improve outcome and quality of life in this group of patients and maintain treatment goals. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Introduction: Sickle Cell Disease (SCD) is a hemolytic disorder with an increased risk of venous thromboembolism (VTE). By the age of 40 years around 11-12% of sickle cell disease patients will have at least one episode of VTE. VTE among patients with SCD is associated with a two to four times increase in mortality compared to SCD patients without VTE. Nevertheless, the evidence guiding VTE management in SCD, specifically in terms of anticoagulant choice, is scarce. Therefore, we conducted a systematic review that evaluates the effectiveness and safety of direct oral anticoagulants (DOACs) in SCD with VTE. Methods: We performed a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the English literature (PubMed, SCOPUS, and Google Scholar) for randomized controlled trials, observational studies, reviews, case series, and case reports for patients with SCD treated with DOAC for thromboembolic disease. We used the terms in combination: "Sickle cell disease" or "Sickle cell anemia", and "DOAC", "rivaroxaban", "apixaban", "dabigatran" "edoxaban". The search included all articles published up to 20th April 2021. Quality and risk of bias assessment were done by two independent authors for each included study. Results: A total of 7 articles were included; four observational studies, and three case series addressing this matter. Patel A et al. found that the use of DOACs, including rivaroxaban, dabigatran, and apixaban in comparison to vitamin K antagonists (VKAs) and low molecular weight heparin (LMWH) for the treatment of VTE in SCD among adults was associated with similar VTE recurrence rate and a better safety profile in terms of a significant reduction in major bleeding events. Similarly, Roberts MZ et al. reported that the use of DOACs for VTE treatment in SCD compared to VKAs resulted in similar effectiveness in terms of VTE recurrence, but the use of DOACs was associated with a similar safety in comparison to VKAs in contrary to the results reported by Patel A et al. in their retrospective study. With regards to the risk of major hemorrhagic events associated with the use of non-VKAs, Gupta VK et al. showed that among 55 patients with SCD treated with VKAs, DOACs, or injectable anticoagulants, only patients treated with VKAs had major bleeding events. Discussion: The current data demonstrated that the use of DOACs for VTE in SCD has similar effectiveness in the prevention of VTE recurrence in comparison to other anticoagulants, including VKAs and injectable anticoagulants with a better safety profile. However, given the absence of clinical practice guidelines for the treatment of VTE among patients with SCD, the clinical practice guidelines recommendations for VTE treatment can be applied to patients with SCD. According to the latest CHEST guidelines (2016) for the treatment of VTE, the use of DOACs is recommended in patients with VTE over VKAs. Similarly, the latest American Society of Hematology (2020) guidelines for VTE suggest the use of DOACs over VKAs, except among patients with renal insufficiency (creatinine clearance less than 30 mL/min), moderate to severe liver disease, or those with antiphospholipid syndrome. Conclusion: In view of the current evidence and based on the results observed; using DOACs was associated with lesser bleeding incidence and fewer complications comparing to VKAs. We think it is rational to use DOACs for VTE treatment among patients with SCD rather than use VKAs. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Introduction Fertility is a complicated subject, it involves more than one individual, and it has profound psychological and economic implications. Moreover, it is affected by several factors, including age, presence of systemic disease, exposure to environmental toxins, medications, or radiation. Compared to the general population, fertility in patients with malignancy is a more complex topic. Cancer survivors, both male and female, may have reduced fertility due to cancer itself or the treatment received. This includes patients with Myeloproliferative neoplasms (MPN), especially that more patients are diagnosed at younger age and patients had good survival and quality of life. As a result, questions regarding fertility and fatherhood are rising, and the effect of the disease and treatment on male fertility is a big concern. There are limited studies that assessed fatherhood in Philadelphia-negative myeloproliferative neoplasms. Methods This is a single-center, mixed-design study (retrospective + phone interviews) conducted within the National Center for Cancer Care and Research. The aim is to evaluate fertility in the Philadelphia-negative MPN male patients and the effect of treatment received on male fertility and the outcome. Inclusion Criteria: Male patients, adult male patients aged >=18 years old, diagnosed with Philadelphia negative MPN (ET PV MF PMF) According to 2008 2016 WHO criteria and actively receiving treatment including tyrosine kinase inhibitors including (hydroxyurea, interferon, and ruxolitinib ) with the following: -patients with no previous fertility problems will be included in the study. -Patients with no known issues with regards to fertility (fertility is intact) will be included in the study. -Patients who developed fertility issues after diagnosis of Philadelphia negative MPN (ET PV MF PMF), who has been evaluated by an andrologist, and evaluation concluded it is related to treatment. Exclusion criteria: - Patient with Philadelphia positive MPN - Patients not fulfilling inclusion criteria are as follow: -Patient was known to have infertility before the diagnosis of Philadelphia-negative MPN. -Patient with infertility after Diagnosis of Philadelphia negative MPN (ET PV MF PMF): If a Clear underlying cause of infertility is not related to treatment, participants will be excluded from the study.if no evaluation was done for infertility or no clear cause for infertility, the patient will be excluded.The mother has documentation by gynecologist for infertility, or after examining the abortion, stillbirth or IUFD and checking the chromosomal analysis (any mother-related cause, whether endogenous or exogenous)will be excluded. Results: Of 120 patients interviewed, only 19 patients (15.7%) had met the inclusion criteria (Figure 1). The majority of patients had lost follow-up or cannot be contacted, and 29.1% of patients had their families completed by the time of diagnosis. The treatment received includes hydroxyurea, interferon, and ruxolitinib. The mode of delivery was normal vaginal delivery in 68% of the pregnancies. The total number of conceptions was 27; three stillbirths were reported; one intrauterine fetal death and one baby of a twin died in utero (table 1). Discussion and Conclusion: A significant percentage of patients diagnosed with Ph-negative MPN are young; the mean age of patients meeting the inclusion criteria at the time of diagnosis was 35 years. This means that a large number of patients are in the reproductive age group or are sexually active. Having a diagnosis of MPN will put these patients in huge psychological distress due to the unknown outcome about their fertility and sexual life and the fear of possible consequences on their children. The data showed that most MPN male patients on treatment had their offspring born normally with no delivery complications, no reported congenital anomaly or growth retardation, and no report of MPN-related cancers. Though, further studies with a larger sample size are required to fully understand the effect of medications on the outcome of fatherhood Philadelphia negative MPN patients. Nonetheless, a call for attention for better education to patients starting on medications addressing the possible psychological fear or concerns of having an unsatisfactory effect on their fertility/offspring, targeting better acceptance and adherence to treatment. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Background: Philadelphia negative Myeloproliferative neoplasms classically characterized by excess production of terminal myeloid cells in the peripheral blood. Among this group, primary myelofibrosis is the least common and usually carries the worst prognosis. Bone involvement in primary myelofibrosis has many forms and tend to manifest as osteosclerotic lesions in vast majority of cases, however osteolytic lesions are reported in exceptional occasions. In this review, we tried to shed the light on this rare association. Methods: We performed a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the English literature (Google scholar, PubMed, and SCOPUS) for studies, reviews, case series, and case reports about patient with myelofibrosis who develop lytic bone lesion. We used the terms in combination: "Myelofibrosis'" or "Primary myelofibrosis" OR "chronic idiopathic myelofibrosis" OR "agnogenic myeloid metaplasia" and "Osteolytic bone lesion", "Osteolytic lesion", "lytic bone lesion". The review included patients with primary myelofibrosis confirmed by biopsy. The reference lists of the included studies were scanned for any additional articles. The search included all articles published up to 10th April 2021. Two independent reviewers screened the titles and abstracts of the records independently and papers unrelated to our inclusion criteria were excluded. A total of 13 articles were included in the review. Results : Total of 13 patients were included in the review. 7 patients were males, male to female ratio almost of 1:1. The mean age at time of diagnosis was 57.69 year, only two cases were diagnosed at young age, however the majority have osteolytic bone lesion at age above 50 years (12/13) of cases. The mean time between the diagnosis of primary myelofibrosis until the osteolytic bone lesion capturing was approximately 8.8 years. 9 out of 13 patients have painful bone lesion, others were incidental finding during a scan for other reasons. All patients have significant splenomegaly. All patients had the lytic lesion detected on x ray, and 2 patients had confirmed findings on magnetic resonance imaging (MRI). The most common affected bones were the vertebrae, pelvis, ribs, humerus then the scapula, femur and skull and less frequently wrist bones and calcaneus. Only one case has reported involvement of the tibia and fibula. The shape, the extension and the numbers of lesion were variable, some showed cortical sparing and others come with cortical destruction. 10 out of 13 cases have confirmed the nature of the osteolytic lesion containing hematopoietic stem cells with or without fibrosis, 2 cases were positive for JAK2 mutation. 2 patients have received ruxolitinib, one of them preceded with bone marrow transplant, others received nonspecific therapies. Discussion: The hyperdynamic ineffective bone marrow can have a negative impact on the bone structure resulting in different types of bone pathology including lytic and sclerotic lesions. The exact mechanism beyond developing lytic lesions is not fully studied, observations revealed two possible causes: systemic inflammation and direct mechanical compression from para-osseus lymph nodes. Lesions prevalence was equal in both genders which can be attributable to a small sample size, in addition, most of the patients were in advanced stages when the lytic lesions discovered and this observation can be explained by the needed time to generate extramedullary hematopoiesis and its subsequent effect on bone structure. The variation in time between the diagnosis of PMF and development of osteolytic bone lesions could be due to the indolent phase of the disease, in which patients can survive for decades without symptoms. Until recently the treatment of myelofibrosis was supportive, but after establishing the JAK2-stat pathway role in myeloproliferative disorders the FDA approved ruxolitinib a JAK2 inhibitor which shows not only survival benefit but also has a significant impact on the resolution of the lytic bone lesions as well. conclusion Osteolytic bone lesions in patients with primary myelofibrosis is extremely rare finding, and noticed shortly after diagnosis in elderly and after longer duration in young patients. The lytic lesion seems to have a bad prognostic value as we can notice 11 out of 13 patients died within one year of detection. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Case report: The main strategy to control the SARS-CoV-2 pandemic is through global vaccination. One of the rare side effects of vaccination is Immune Thrombocytopenic Purpura (ITP). We present a 31 years old lady with a history of ITP, came on her 8th week of pregnancy with fever and dry cough after receiving the first dose of Pfizer vaccine. The ITP flare worsened after the second dose of the vaccine. Patients with ITP should have their second dose of vaccine delayed if they had flare particularly if pregnant.

Introduction Chronic myeloid leukemia is a myeloproliferative neoplasm (MPN) that is characterized by the uncontrolled production of mature and maturing granulocytes, mainly neutrophils. The molecular defect in CML is related to the reciprocal translocation between chromosomes 9 and 22, resulting in the BCR-ABL1 fusion gene. Typically, CML is BCR-ABL1 positive. Mutation like JAK2, which is seen in BCR-ABL1 negative MPN is usually absent in CML. However, there is an uncommon entity of CML which has been reported to have both BCR-ABL1 positive and JAK2 mutation concomitantly. Little is known about the characteristics of the best treatment modalities and the outcome of CML with double-positive ( JAK2 and BCR-ABL1). Methods We searched the English literature (Google Scholar, PubMed, and SCOPUS) for studies, reviews, case series, and case reports of patients with chronic myeloid leukemia who had JAK2 mutation. Inclusion criteria: were the presence of JAK2 mutation in CML patients with BCR-ABL1 rearrangement and, secondly, age ≥18yrs. The search included all articles published up to 20th April 2021. Results and Discussion A total of 25 patients met our criteria of search. Twenty-two patients were diagnosed in the chronic phase, 2 patients in the accelerated phase, and one patient transformed to the blast phase. More females n=16 and 10 males. The mean age at the time of diagnosis was 61.3 years. 9 patients had to be switch to a second-line therapy. 7 patients were treated with imatinib; only 1 patient has loss of JAK2 mutation with imatinib (1 out of 7). While 3 patients treated with dasatinib 2 responded, and one 1 had persistent JAK2 mutation. The reason for detecting the JAK2 mutation was persistent thrombocytosis or erythrocytosis, failure to respond to treatment, or suspected other MPN. Hematological parameters appear close to other CML; patients with mean WBC 56.8 x10^3/uL, mean PLT of 541.9 x10^3/uL, and mean Hb of 12.4 g/dl. The characteristics of age and gender for this category of patients with CML are not significantly different from other CML patients. Age and gender distribution and the presence of splenomegaly or organomegaly are almost the same. Males were slightly more than females as CML has a slight male predominance. The mean age was 58 years, which is close to the mean age of CML patients. From the data available, most patients treated with dasatinib had the JAK2 mutation undetectable on follow-up, while patients on imatinib JAK2 mutation persisted despite that some had CML in remission. This might means that the driving mutation is probably BCR-ABL. Regarding the response to the TKI 3 patients who had dasatinib 2 had a loss of JAK2 and one JAK2 persisted. Only one patient on imatinib had a loss of JAK2 out of 7. The reason behind the high success rate of achieving remission with medication like dasatinib could be explained by the drug's dual tyrosine kinase inhibitors activity. Imatinib acts by inhibiting the BCR-ABL1 pathway, inhibiting the tyrosine kinase by competitive inhibition, blocking the ATP binding site, and thereby preventing the conformational switch to the active form [0]. In comparison, a broad-spectrum kinase inhibitors (less selective) like dasatinib is (a second-generation tyrosine kinase inhibitor) inhibiting the BCR-ABL1 like imatinib, and additionally, they inhibit other signaling pathways like SRC kinases. This may allow multikinase inhibitors to have additional therapeutic effects that can include the JAK2 mutation. Patients with BCR-ABL-JAK2 Positive Chronic Myeloid Leukemiahave the same characteristics of BCR-ABL CML, and also they share some features like persistence of thrombocytosis. Conclusion It is difficult to conclude that multi-kinase inhibitors are superior to imatinib in treating CML with concomitant JAK2 mutation. But the result of the reported cases showed that multi-kinase inhibitors are more likely to be successful in achieving remission and loss of JAK2 mutation. However, it is difficult to generalize the result without further studies due to the few numbers of patients and the unusual association. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Objective To determine the effect of interpregnancy interval (IPI) on obesity/overweight among pregnant Sudanese women in early pregnancy. Methods A cross-sectional study was conducted at Saad Abu-Alela Hospital, Khartoum, Sudan, from January to December 2015. Women with a singleton pregnancy and at least one previous pregnancy were enrolled in the first trimester. A questionnaire was used to collect clinical and sociodemographic data from the participants, and current body mass index (BMI) was calculated as weight in kilograms divided by the square of height in meters. Results Among 1045 participants, 344 (32.9%), 424 (40.6%), and 277 (26.5%) women had short ( 30 months), respectively. BMI indicated 18 (1.7%), 408 (39.0%), 381 (36.5%), and 238 (22.8%) women were underweight (

Background: The association between the haematological profile (including abnormal platelets) and malaria is not completely understood. There are few published data on haematological profiles of malaria patients in areas with unstable malaria transmission. The current study was conducted to investigate if the haematological parameters, including platelet indices, were reliable predictors for microscopically-diagnosed malaria infection. Methods: A case-control study with a total of 324 participants (162 in each arm) was conducted at the out-patient clinic of New Halfa hospital during the rainy and post rainy season (August 2014 through to January 2015). The cases were patients with uncomplicated Plasmodium falciparum (107; 66.9%) and P. vivax malaria (55, 34.0%) infections. The controls were aparasitemic individuals. The haematological parameters were investigated using an automated hemo-analyser. Results: There was no significant difference in the mean (±SD) age between the study groups; however, compared to the controls, patients with uncomplicated malaria had significantly lower haemoglobin, leucocyte and platelet counts, and significantly higher red cell distribution width (RDW), platelet distribution width (PDW) and mean platelet volume (MPV). Conclusions: The study revealed that among the haematological indices, PDW and MPV were the main predictors for uncomplicated P. falciparum and P. vivax malaria infection. Abbreviations: OR: odds ratio.

Background: The exact influences of thyroid functions on body mass index (BMI) are ill-defined in euthyroid pregnant women with gestational diabetes mellitus (GDM). Objectives: To investigate the effect of GDM on maternal thyroid functions and BMI. Methods: A case- control study was conducted in Saad Abualila Hospital, Khartoum, Sudan June to August 2015. Cases included women with GDM and healthy pregnant women as controls. Thyroid hormones [thyroid-stimulating hormone (TSH), free tri-iodothyronine (FT3), and free thyroxine (FT4)] and anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibodies were measured. Results: BMI was significantly increased in GDM patients (26.3 (2.7) Kg/m2) compared with the control group (24.3(1.8) Kg/m2, P = 0.001). Levels of FT3 and FT4 were significantly decreased in GDM patients (0.632 (0.408 ─ 1.074) pg/ml; 0.672 (0.614 ─ 0.960) ng/dl) compared with the healthy pregnant women (0.820 (0.510─1.385) pg/ml, P = 0.021; 0.840 (0.767─1.200) ng/dl, P < 0.001). In contrast, anti-TPO and anti-TG were significantly higher in GDM patients (11.13 (7.969 ─13.090) IU/ml; 14.40 (10.91─20.69) IU/ml) compared with the control group (8.90 (6.375─10.48 IU/ml, P = 0.022; 10.50 (8.2─13.95) IU/ml, P = 0.010). BMI correlated negatively with FT3 (r = ─ 0. 375, P = 0.002) and FT4 (r = ─ 0. 316, P = 0.009) and positively with anti-TPO (r = 0.361, P = 0.002) and anti-TG (r = 0.393, P = 0.010). Conclusion: The present results add further evidence for decreased free thyroid hormones, increased anti-thyroid autoantibodies and higher BMI in patients with GDM compared to healthy pregnant women. BMI correlated directly with FT3 and FT4, but failed to demonstrate significant association with TSH.