Spontaneous spinal extradural hematomas are rarely attributed to hemophilia. When it occurs, magnetic resonance imaging (MRI) best establishes the diagnosis that should prompt immediate medical or surgical management. Here, we present a case with large spinal epidural hematomas (EDH) which was managed conservatively with good neurological outcome. A 12-year-old male who is a known case of hemophilia A was admitted with a complaint of neck pain, was neurologically intact on examination with no motor or sensory deficits. MRI cervical spine revealed a large spinal EDH in the cervical region compressing the cord posteriorly. The patient was managed conservatively with tranexamic acid and factor VIII replacement and observed over few days. He recovered well with interval resolution of hematoma. this case depicts the role of conservative management of spontaneous EDH in patients with Hemophilia A utilizing factor replacement therapy, steroids, and tranexamic acid and emphasizes that surgery is not always required even in such radiologically large appearing lesions.

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BACKGROUND: Coronavirus disease 2019 (COVID-19) pandemic has caused disruption of oncology services, but data on actual delays are lacking. We sought to study the delays faced by the cancer patients during the COVID-19 pandemic and the impact of measures carried out to reduce the delays. METHODS: We carried out a review of inpatient and outpatient oncology services from March 15 to June 15, 2020, at our institution. We obtained data on delays in referrals, diagnosis, chemotherapy, and treatment delivery for cancer patients. To examine the delays, patients were assessed in the inpatient and outpatient settings and according to cancer types. RESULTS: A total of 505 patients' events were included in the study, with 136 referral delays, 167 diagnostic delays, 121 chemotherapy delays, and 81 treatment delays. In the outpatient settings, maximum referral delays were faced by breast cancer patients (9.5 ± 3.3 days, range 7–14), followed by hematologic malignancy (hematology) patients (8.4 ± 4.5 days, range 0–14). Maximum diagnosis delay was faced by hematology patients (11.75 ± 5 days, range 0–14), followed by breast cancer patients (11.2 ± 3.4 days, range 7–14). Breast and colorectal cancer patients faced maximum chemotherapy delays (9.5 ± 6.6 days, range 0–14), followed by hematology patients (7.3 ± 6.6 days, range 0–14). In the inpatient setting, maximum diagnosis delays were faced by hematology patients (11 ± 2 days, range 10–14), followed by gastrointestinal cancers (8 ± 2.4 days, range 5–10). Maximum chemotherapy delays were faced by hematology patients (8.5 ± 5.9 days, range 0–14) and breast cancer patients (8 ± 2.4 days, range 5–10). Hematology patients were most affected by treatment-delays (6.75 ± 4.7 days, range 0–10). Comparison of delays during the earlier part of the study with the later part showed that referral delays improved by 3.5 days (P = 0.02), diagnosis delays improved by 6.1 days (P < 0.001), chemotherapy delays improved by 7.7 days (P < 0.001), and treatment delays improved by 8 days (P < 0.001). CONCLUSIONS: COVID-19 pandemic caused delays in the diagnosis and management of most cancer patients. Measures adopted to overcome delays resulted in a significant reduction in delays.

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BACKGROUND: Automated hematology analyzers effectively reduce turnaround time and generates scattergrams which can be used to screen for different hematological conditions. AIMS: We wish to study the scattergrams generated by the Sysmex XN-series analyzer for confirmed cases of hematological malignancies and evaluate for the specific patterns to these malignancies. METHODS: Two hundred and ninety-one newly diagnosed cases of various hematological malignancies were included in the study. All these cases were diagnosed and classified in accordance with flow cytometry, bone marrow morphological study, immunohistochemistry, and molecular studies. Forty-eight cases of leukemoid reaction were also included in the study. We retrieved the scattergrams and complete blood count data of all these cases which were processed in the Sysmex XN-analyzer before their diagnosis. Along with scattergrams, the peripheral blood smear findings of all the cases were extracted too. The scattergram patterns in the white blood cell differential (WDF) and white cell nucleated region (WNR) channels were evaluated and correlated with their peripheral blood smear findings. RESULTS: All the scattergrams in the WDF and WNR channels were studied. Patterns seen in acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) were striking and distinctly separate. After correlating with the peripheral smear and immunophenotyping, AML patterns were subtyped by the French American British classification. Acute promyelocytic leukemia as well as different phases of chronic myeloid leukemia had unique patterns. Scattergram patterns of ALL had minute difference with that of chronic lymphoproliferative disorder. CONCLUSION: In high-volume laboratories, the patterns on Sysmex XN-series analyzer scattergrams can be used as a valuable aid for suspecting and triaging cases of hematological malignancies.

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Progressive pallor with hepatosplenomegaly is an alarming constellation of signs in children indicating a serious, ongoing disease process which has to be investigated for. Anemia and hepatosplenomegaly can be caused by a wide spectrum of conditions ranging from benign to life-threatening ones. We report the case of a 4-year-old child who presented with progressive pallor and hepatosplenomegaly along with diagnostic approach and review of literature. The child was diagnosed to have Gaucher disease.

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Systemic mastocytosis (SM) characterized by focal or diffuse infiltrates of neoplastic mast cells can range from indolent cutaneous disease to aggressive systemic disease. Skin involvement is usual in indolent systemic mastocytosis, less frequent in aggressive SM, and rare in mast cell leukemia. Here, we present a rare coexistence of cutaneous mastocytosis and SM with an associated hematologic neoplasm SM-associated hematologic neoplasm [AHN] (atypical chronic myeloid leukemia [CML]-BCR/ABL negative), the first case being reported in the literature so far to the best of our knowledge. An elderly male with leukocytosis, myeloid hyperplasia, and mast cell infiltrates in the marrow was diagnosed with atypical CML and cutaneous mastocytosis. Approximately 40% of cases of SM are associated with a hematologic neoplasm (SM-AHN) encompassing all defined myeloid neoplasms, such as myelodysplastic syndrome, myeloproliferative neoplasm, acute myeloid leukemia, CML, chronic myelomonocytic leukemia, or plasma cell myeloma. The prognosis of SM-AHN is dependent on the associated hematologic neoplasm (AHN).

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Since its discovery in 1953, the presence of the antigen Diego (Di) has been related to the Mongoloid populations and American natives. Given its immunogenic potential, the presence of the mentioned antigen is related to the development of hemolytic disease of the newborn (HDN). We present the case of a puerperal patient with no known history who was detected the presence of antibodies to Di b during study of a mild HDN presented by her daughter. When studying the patient's family, the presence of Dib antigen was reported in a brother as well as in both parents. We consider that being a rare event, this type of case should be reported promptly to promote the exchange of knowledge with other health professionals and the community.

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BACKGROUND: Peripheral autoimmune cytopenia is defined by the immune-mediated destruction of hematological cell lines, including platelets, red blood cells, white cells (neutrophils), and multi-line destruction, called Evans syndrome. METHODS: Our retrospective study includes 59 patients admitted to the hematology service in Constantine Regional Military University Hospital during a period of 10 years (2010–2019) with the aim of specifying the epidemiological, clinical and biological characteristics as well as the characteristics therapy of adult autoimmune cytopenia in eastern Algeria region. RESULTS: Through this study, it can be noted that immunological thrombocytopenic purpura (ITP) is the most frequent of cytopenia 79.33% (47 cases) followed by autoimmune hemolytic anemia (AHAI) by 20.34% (11 cases). The average age for ITP is 52.04 ± 5.78 years with a male predominance and a gender F/M ratio of 0.81. People over the age of 60 were the most affected. The diagnosis is evoked in the presence of a hemorrhagic syndrome in 51.06% of cases. Thrombocytopenia (platelet count <100 g/l) is found in all our patients. Of the 24 cases with hemorrhagic syndrome, 83.33% have thrombocytopenia <30 g/l. The average age for AHAI is 46.92 ± 10.16 years with a gender F/M ratio of 0.71. The diagnosis is evoked in the presence of an anemic syndrome in 91.66% of cases. Anemia is found in all our patients, with an average hemoglobin level of 7.56 ± 0.95 g/dl. Direct positive test in 66.67% of cases. The markers of hemolysis remain mainly disrupted by the haptoglobin which is reduced with an average rate of 0.06 g/l. CONCLUSION: Peripheral autoimmune cytopenia is rare; we can remember that in our context ITP is the most frequent of autoimmune cytopenia in adults. It's a pathology that affects both sexes with a male predominance. In the AHAI, we have noticed a slight male predominance. Its main clinical manifestation is anemic syndrome and corticosteroids are the first-line treatment in both types ITP and AHAI.

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INTRODUCTION: Differential diagnosis of hemoglobin (Hb) variants eluting in the A₂ window on high-performance liquid chromatography (HPLC) is of particular importance. Of particular importance is Hb E, which is the most common and the most significant variant. The aim of this study was to study a rare variant infrequently seen in our countries during the routine work. METHODS: Sixteen Bengali workers at Najran University Hospital, Saudi Arabia, came to the laboratory for routine investigation. CBC, routine blood chemistry, and Hb separation by HPLC were performed. RESULTS: Three cases out of 16 showed an abnormal Hb peaked on the A₂ window on HPLC consistent with the diagnosis of Hb E. Two of them had Hb E of 29.1% of each. The third case had Hb E around 86%. In addition, one case was consistent with β thalassemia trait had increased Hb A₂ of 5.7%. Five cases were borderline. CONCLUSION: HbE could be adequately differentiated from other Hb variants eluting in the A₂ window on HPLC by the percentage of the variant, its retention time, the mild clinical presentation, and the ethnic origin of the patient.

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OBJECTIVES: The objective of the study was to address parents' perspectives and to investigate contributing factors that led to suboptimal results of premarital screening for sickle cell anemia (SCA) or thalassemia, as nearly 50% of at-risk couples decline the advice they are given and instead proceed with the marriage. METHODS: A cross-sectional study of forty parents (mother or father) who were identified as being in an at-risk couple based on results of premarital screening for carriers of SCA or thalassemia was conducted. These at-risk couples ignored medical advice, proceeded with marriage, and subsequently gave birth to a child afflicted with SCA or thalassemia. The study was composed of a self-administered questionnaire with scenario-based and advice-seeking questions from the point of view of a relative or friend in a similar marital situation. RESULTS: Results of this study found that parents of children with SCA or thalassemia advised against proceeding with an at-risk marriage. Most of the participants felt pressured to continue with their marriage, as premarital testing was conducted too late; furthermore, they felt guilty about their decision and wished that the marriage had not occurred. CONCLUSION: This study highlights some of the current drawbacks of premarital testing when it is conducted very close to the wedding, which makes it very difficult to stop an at-risk marriage, as this is socially unacceptable.

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Many uncertainties exist regarding COVID-19 vaccination in patients undergoing treatment for hematological malignancies. We intend the illustrate the various types of COVID-19 vaccine currently in use and their mechanism of action. We have complied, recommendations for COVID-19 vaccination in patients suffering with specific hematological malignancies and those undergoing HCT and CAR-T cell therapy in this review. We have also discussed the available safety data for COVID-19 vaccination in the immunocompromised population.

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Coronavirus disease-19 (COVID-19) is an ongoing global pandemic with approximately 15% of patients requiring mechanical ventilation and intensive care unit care. The mechanisms of end-organ damage and mortality in severe COVID are slowly being unraveled. COVID-related coagulopathy is a characteristic syndrome that plays a key role in multi-organ dysfunction and severe disease. Mechanistically, many components including endothelial cells, the coagulation system, complement system, cytokines, and NETosis are involved in the pathogenesis of this syndrome and provide potential therapeutic targets. We provide a focused review on the current understanding of COVID-19-related coagulopathy and its therapeutic implications.

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