Acute Leukemia in Niger: Epidemiological, Diagnostic and Therapeutic Aspects

Objective: Improve the care of patients followed for acute leukemia in the Oncohematology department of the National Hospital of Niamey. Methods: This was a prospective study, over a period of 2 years from January 1, 2018 to December 31, 2019, in patients with acute leukemia in the Oncohematology department of the National Hospital of Niamey (HNN), whose diagnosis was made on a blood smear associated with a myelogram and immunophenotyping and who were consenting. Results: We collected 25 cases of acute leukemia confirmed by myelogram and immunophenotyping. The mean age of the patients was 31.32 years, with a predominance of women, a sex ratio of 0.92. Pupils and students were in the majority with 40% and most came from the Niamey region, i.e. 68%. Anemic syndrome was the most common clinical sign in 96%. ALL predominated in 64% of cases. On the blood count, the hyperleukocytosis was more marked in AML (mean white count: 197256.6 elts/mm3) than in ALL (137891.6 elts/mm3), it was the same for thrombocytopenia which is more marked in AML (75588.89/mm3) than in ALL (52156.25/mm3). Therapeutically, 52% of patients received chemotherapy. The mean overall survival was 16.223 ± 3.191 months, including a mean survival for AML of 6.853 ± 1200 months compared to 21.720 ± 5.920 months for ALL. Conclusion: Acute leukemia still remains a major problem in our context, due to the precariousness of limited financial, diagnostic and therapeutic resources. Thus reflecting in our results, the increasing number of cases, the diagnostic delay and the guarded prognosis. This is the reality in several other countries in the sub-region and even in certain developed countries.

Primary Non-Hodgkin’s Malignant Lymphoma of the Uterus at the Reference Hospital of Maradi/Niger: A Case Report

Malignant non-Hodgkins lymphoma (MHNL) of the uterus is uncommon. We report a case diagnosed on the basis of histologic and immunohistochemical studies of a hysterectomy specimen induced by a very painful pelvic mass in a 50-year-old patient with no previous history of the disease. It was classified as Ann Arbor IV Bb after imaging, given the medullary infiltration and signs of clinical and biological evolutivity: the patient had received two courses of chemotherapy, CHOP protocol. She died 23 days after the second treatment due to a hypertensive crisis.

Pulmonary and Orbital Bifocal IgG4 Disease: Case Report and Literature Review

Introduction: IgG4 disease is an orphan, fibro-inflammatory autoimmune disease of recent discovery whose thoracic involvement is rarer. We report a case of Pulmonary and orbital bifocal IgG4 disease with a review of the literature. Observation: This is a 71-year-old patient with a history of hypertension, dyslipidemia, smoking cessation at 15 PA, with progressive dyspnoea, weight loss of 4 kg with PS = 0 for 2 months. The thoracic CT scan revealed 3 pseudotumoral lung lesions of the LIG, LID and LM. The histology of the two CT-guided lung biopsies and the LIG wedge had objectified inflammatory lesions without signs of malignancy. The evolution was marked by the occurrence of a right orbital edema. The cerebral scanner found a voluminous right orbital inflammatory pseudotumor. Biopsy with histology found fibroinflammatory lesions with lymphoplasmacytic infiltrates and positive immunolabeling with anti-IgG4 antibodies. The PET scanner had objectified pulmonary and pleural parenchymal consolidations and moderately hypermetabolic mediastinal ADP with max SUV between 3 and 6. The patient was put on corticosteroid therapy with a favorable outcome. Conclusion: IgG4 disease is rare and difficult to diagnose despite well-defined and consensual diagnostic criteria and classification. The discovery of new biomarkers facilitates the diagnosis and monitoring of patients. Well-codified corticosteroid therapy is effective but possibility of recurrence. The current challenge remains the lack of data on the follow-up of these patients to assess the risk of neoplasia (lymphoma).

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.

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.

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.