Alternative Phenotypic Profile for B-Cell Abnormality in a Case at Zinder National Hospital

Introduction: Since it is impossible to establish a diagnosis in the presence of hyperlymphocytosis not secondary to lymphocytic hyperactivation, we considered a B-lymphoid hematopathy with a non-specific phenotypic profile. We report one case of this. Observation: This is a forty-eight (48) year old patient with hyperlymphocytosis at 139,000 elements per cubic millimeter, heterogeneous splenomegaly at 25.6 cm in diameter on abdominal ultrasound without detectable deep or peripheral lymphadenopathy. Peripheral blood cytology shows lymphocyte cell proliferation suggestive of the circulating phase of chronic lymphoproliferative B syndrome. The expression profile of cell membrane markers did not allow for the definition of a specific phenotypic profile. Faced with this immunophenotyping result, we considered a B-lymphoid hemopathy with a non-specific phenotypic profile. After three courses, the MINICHOP treatment was used to achieve partial remission with wasting of more than 80% of the evaluable masses. Conclusion: Despite the contribution of immunophenotyping in the diagnosis of lymphoproliferative syndromes, it is possible to consider the diagnosis of a B-lymphoid hemopathy with a phenotypic non-specific profile of CD45+, monotypic kappa, CD19+, FMC7+, CD22+, CD5−, CD79b−, CD23−, CD43−, CD38−, CD200−.

Sickle Cell Disease in the Zinder Region in 2023: Prevalence and Sociodemographic Factors

Introduction: In view of the number of sickle cell patients and due to a low production of descriptive studies, we decided to determine the prevalence of genes S and C of the disease in the Zinder region. The objective was to contribute to improving the management of sickle cell disease in Zinder. Methodology: This was a systematic screening by the “Sickle Scan” test of any blood donor admitted to the Zinder Regional Blood Transfusion Center during the 6-month study period, from January to June 2023. The Sickle Scan is a qualitative lateral flow chromatography immunoassay using whole blood samples that aid in the rapid diagnosis of sickle cell disease. Results: The study was carried out on 613 samples during the period concerned. The frequency of sickle cell genes was 26.9% (n = 165) in all samples collected, with 23.1% (n = 142) and 3.8% (n = 23) for the S gene and the C gene, respectively. The 18 - 30 age group was the most represented with 64.4% (n = 395) cases. The median age of blood donors was 26 years ± 10 years (min = 18 years/max = 60 years). The sex ratio was 2.5. Donors of Nigerien nationality accounted for 84.1% (n = 516). There is a predominance of blood donors with an average monthly income between 34,000 and 70,000 CFA francs in 44.3% (n = 272), lived in permanent housing with drinking water supply. Sickle cell trait (SMA) was found in 22.5% (n = 138). Conclusion: The analysis of these results highlights a high frequency of the S gene for sickle cell disease. The population with an average monthly income is the most affected, with a male predominance.

Deep Vein Thrombosis Complicating SS Sickle Cell anemia in Pregnancy: About a Case Diagnosed at the Issaka Gazobi Maternity

Pregnancy in a sickle cell patient is a source of maternal and perinatal morbidities. Thromboembolic diseases of which Deep Vein Thrombosis (DVT) is one of the complications. The frequency of the latter is poorly assessed. Very few studies have been done on this topic. We report a case of DVT in a 35-year-old patient with sickle cell SS pregnant with 27SA diagnosed and cared for at the Maternity Issaka Gazobi (MIG) in Niamey. The management was based on low molecular weight heparin combined with preventive measures against sickle cell anemia and a planned cesarean section which led to a favorable maternal and perinatal prognosis.

First Case of Von Willebrand Disease in Niger

Von Willebrand’s disease was first described in 1926 by Erik Von Willebrand. It is a genetic, constitutional defect of hemostasis that is different from hemophilia. It is classified among the rare diseases whose clinical manifestations are dominated by a hemorrhagic profile, which varies from patient to patient. It is an easily diagnosed disease based on a quantitative and qualitative bioassay of VWF. Treatment is multidisciplinary and is based on well-structured prevention. We report the first case of von Willebrand disease in Niger, diagnosed in the Hematology Department of Niamey’s National Hospital.

Chronic Lymphocytic Leukemia of del 17p in a Young Subject: About a Case and Reviewed a Literature

Objective: To report a case of Chronic Lymphoid Leukemia in a 28-year-old young subject, with variable clinical features and a TP53 mutation, diagnosed and followed up in the Onco-Hematology department of the HNN. Observation: 28-year-old patient, having consulted for polyadenopathy and physical asthenia, whose clinical examination found a conscious patient, submaxillary, laterocervical, axillary and inguinal lymphadenopathy, bilateral, symmetrical, painless and non-compressive whose largest measures 3 cm in diameter. Hepato-splenomegaly and epistaxis. Predominantly lymphocyte hyperleukocytosis, immunophenotyping revealed low CD19+, CD5+, CD23+, CD20 monoclonal B lymphoid proliferation. The Matutes score was 4. A karyotype showed a three-chromosome translocation;the short arm of a chromosome 2, the long arm of a chromosome 11 and the long arm of a chromosome 13, and a translocation between the long arm of a chromosome 6 and the long arm of a chromosome 18. A FISH objectified a led 17p. The diagnosis of Binet Stage C CLL with positive del 17p and complex karyotype was retained. Despite the poor prognosis, the R-C (Rituximab-Chlorambucil) protocol was instituted with once-weekly transfusions. The patient is still alive in partial clinical and biological remission. Conclusion: Despite therapeutic progress, the presence of the deletion of chromosome 17p with TP53 mutation and the young age of the patient does not change the patient’s prognosis.