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

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Open Journal of Blood Diseases 2024年第1期14卷 1-7页

作者： Brah Moustapha Maman amadou djibrilla-almoustapha Moustapha Elhadji-Chefou Illa Hamidine Moussa Diongolé Hassane Badé Malam-Abdou Department of Medicine Université André Salifou de Zinder Zinder Niger Department of Medicine H&#244pital National de Zinder Zinder Niger Department of Medicine Université Abdou Moumouni de Niamey Niamey Niger Department of Medicine Université Dan Dicko Dan Koulodo de Maradi Maradi Niger

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.

关键词： Sickle Cell Anemia Blood Donors Sickle Scan

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

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Open Journal of Blood Diseases 2024年第2期14卷 43-46页

作者： Moustapha Elhadji-Chefou Badé Malam-Abdou amadou djibrilla-almoustapha Maman Brah Moustapha Oumou Kimso Ibrahim Timi Liman Elhadji Ali Issa Abdou amadou Borges Moreno Yuribet Facult des Sciences de la Sant Universit Dan Dicko Dankoulodo de Maradi Maradi Niger Hopital de Reference de Maradi Maradi Niger Facult des Sciences de la Sant Universit Abdou Moumouni de Niamey Niamey Niger Facult des Sciences de la Sant Universit Andr Salifou de Zinder Zinder Niger

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.

关键词： Malignant Non-Hodgkin’s Lymphoma Uterus Referral Hospital Maradi/Niger

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

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Open Journal of Blood Diseases 2024年第2期14卷 59-64页

作者： Abasse Moussa Ounteini Mamane Daou Badé Malam-Abdou Brah Souleymane amadou djibrilla almoustapha Yerima Salifou Seraphin Adjoh Komi Etienne Devin Habeeb Mahmoud Anas Mehdaoui Department of Pneumology and Thoracic Cancerology Eure-Seine Hospital vreux France Pulmonology Department Niamey National Hospital Niamey Niger Department of Internal Medicine General Reference Hospital FSS-UAM of Niamey Niamey Niger Department of Hematology National Hospital of Niamey FSS-UAM of Niamey Niamey Niger Pneumology Department Sylvanus Olympio University Hospital of Lom Lom Togo

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).

关键词： Orphan Disease Thoracic IgG4 Disease Autoimmunity

Acute Leukemia in Niger: Epidemiological, Diagnostic and Therapeutic Aspects

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Open Journal of Blood Diseases 2024年第3期14卷 81-90页

作者： amadou djibrilla-almoustapha Badé Malam-Abdou Abdourahamane Yacouba Moussa Souley Moustapha Maman Brah Moustapha Elhadji-Chefou Boubacar Marou-Soumana Samaila Aboubacar Ousseini Fanta Maman Rabiou Badé Oumarou Adamou-Chaibou Department of Hematology-Oncology National Hospital of Niamey Niamey Niger Faculty of Health Sciences University of Abdou Moumouni Niamey Niger Department of Medicine Zinder Hospital Regional Niger Departtment of Biology Amirou Boubacar Diallo National Hospital Niamey Niger Faculty of Health Sciences University of Dan Dicko Dankouloudo of Maradi Maradi Niger Laboratory of Biology National Hospital of Niamey Niamey Niger

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.

关键词： Acute Leukemia ALL AML Hematology-Niamey National Hospital (HNN) Niger

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

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Open Journal of Blood Diseases 2021年第2期11卷 25-30页

作者： Chaibou-Yacouba Maimouna Hamidou Soumana-Diaouga amadou djibrilla-almoustapha Moctar Abdoulaye-Maliki Mansour Sidi-Mahamane Madeleine Garba-Rah Idi Nafiou Madi Nayama Issaka Gazobi Maternity Niamey Niger Science of Health Faculty Abdou Moumouni University of Niamey Niamey Niger Hematology Department of the Niamey National Hospital Niamey Niger Cardiology Department of the Niamey National Hospital Niamey Niger

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.

关键词： DVT Sickle Cell Disease Gravido-Puerperalitis MIG Niamey

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

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Open Journal of Blood Diseases 2022年第4期12卷 103-109页

作者： amadou djibrilla-almoustapha Moustapha Mamane-Brah Moustapha Elhadj-Chefou Maman Rabiou Badé Faiza Abba-Ousmane Fanta Ousseini Oumoulkairou Abdoulaye-Soumana Badé Malam-Abdou Departement of Onco-Hematology of the National Hospital of Niamey Niamey Niger Faculty of Health Science Abdou Moumouni University of Niamey Niamey Niger Faculty of Health Science André Salifou University of Zinder Zinder Niger Faculty of Health Science of Maradi Maradi Niger

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.

关键词： Chronic Lymphocytic Leukemia Young Subject del 17 p HNN Niger

First Case of Von Willebrand Disease in Niger

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Open Journal of Blood Diseases 2021年第1期11卷 1-5页

作者： amadou djibrilla-almoustapha Moustapha Maman-Brah Moustapha Elhadji-Chefou Maman Rabiou Badé Amal Al-Azhari Youssoufa Seydou-Moussa Maman Aminou Mahamane Sani Mamane Daou Souleymane Brah Badé Malam-Abdou Hematology Department of the Niamey National Hospital Niamey Niger Department of Internal Medicine of the National Hospital of Niamey Niamey Niger Endocrinology Department of the Niamey General Reference Hospital Niamey Niger Department of Internal Medicine of the General Reference Hospital of Niamey Niamey 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.

关键词： Willebrand Disease Onco Hematology Niamey Niger

Pathological and Etiological Aspects of Nephrotic Syndrome at the Niamey General Reference Hospital

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Open Journal of Nephrology 2024年第3期14卷 340-349页

作者： djibrilla Gani Salamatou amadou Niaouro Oumarou Ali Diallo Nephrology-Dialysis Department Niamey General Reference Hospital Niamey Niger Environmental Health and Climate Epidemiology Unit (UESEC)-CERMES Niamey Niger

Introduction: Studies have been conducted on nephrotic syndrome in Niger. The study aimed to determine the histological and etiological aspects of nephrotic syndrome. Patients and Method: This was a retrospective study from February 1st, 2018 to January 31st, 2024. All patients with nephrotic syndrome who underwent renal biopsy were included. Samples were analyzed at the anatomy-cytology pathology laboratory of the Faculty of Medicine in Dakar (Senegal). The variables studied included clinical, biological, histological and etiological characteristics. Data were analyzed using Excel 2013 and Epi-info 7.2.0 software. Results: The study included 119 patients with nephrotic syndrome. Prevalence of nephrotic syndrome was 11.24%. The male-to-female ratio was 2.25:1. The mean age at diagnosis was between 34.5 ± 18.84 years. Edema was the reason for admission in 40.34% of cases. The nephrotic syndrome was impure in 63.86% of cases. Nine histological lesions were identified. Focal and segmental glomerulosclerosis (40.09%), minimal change disease (23.53%), membranous nephropathy (13.45%), diabetic nephropathy (10.92%), membranous proliferative glomerulonephritis (3.36%), acute glomerulonephritis (3.36%), glomerular thrombotic microangiopathy (2.52%), non-IgA mesengial proliferative glomerulonephritis (1.68%) and amyloidosis (0.84%). Nephrotic syndrome was primary in 57.98% of cases. Secondary etiologies were dominated by diabetes (11.76%), followed by hepatitis B virus (9.24%), lupus, lymphoma, malaria, syphilis, cryoglobulinemia, sickle cell disease and HIV. Conclusion: Future studies should investigate the causes of glomerulopathy secondary to chronic tubulointerstitial lesions.

关键词： Nephrotic Syndrome Anatomopathology Niamey General Reference Hospital

Clinical and Bacteriological Profile of Infections in Sickle Cell Children in Two Referral Hospitals in Niamey, Niger

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Open Journal of Pediatrics 2024年第1期14卷 36-42页

作者： Kamaye Moumouni Samaila Aboubacar Garba Moumouni Georges Thomas Ibrahim Mamoudou Abdou Djafar Mamane Halima Hamadou Ibrahim Hamani Issaka djibrilla almoustapha amadou Yacouba Abdourahamane Marou Soumana Boubacar Moussa Saley Sahada Bade Malam Abdou Soumana Alido Service de Pédiatrie A H&#244pital National de Niamey Niamey Niger Faculté des Sciences de la Santé Université Abdou Moumouni de Niamey Niamey Niger Service de Pédiatrie H&#244pital national Amirou Boubacar Diallo Niamey Niger Service d’Onco-Hématologie H&#244pital national de Niamey Niamey Niger Service de Biologie Médicale H&#244pital national Amirou Boubacar Diallo Niamey Niger

Introduction: Infections are significant causes of mortality in sickle cell children in resource-limited countries. This study aimed to determine the clinical profile and bacterial ecology of infections in children with sickle-cell disease in two referral hospitals in Niamey. Patients and methods: A retrospective descriptive study was conducted from January 2018 to July 2020 in two referral hospitals in Niamey. All children aged one (1) to 15 years with sickle cell disease admitted for suspected infection, including at least one bacterial culture, were studied. Bacteriological analysis was performed using the appropriate culture media, using BactAlert (Reference 4700003 BTA3D60 BioMérieux). Results: Over 36-months, 350 children with a mean age of 10.9 months were admitted. The sex ratio was 1.2. The SS electrophoretic profile was the most common (93.4%). Immunization status was up to date in 66% of patients. Fever was the common reason for consultation (55.1%). Infection was confirmed in 62 patients (17.7%). The primary diagnoses were bacterial gastroenteritis (24.2%) and urinary tract infection (19.4%). Blood cultures were isolated from Salmonella typhi (13.0%) and Escherichia coli (8.7%). Klebsiella spp (7.1%) and Escherichia coli (5.0%) were detected in cytobacteriological examination of urine. Salmonella typhi (23.5%) and Escherichia coli (5.9%) were isolated on coproculture. Conclusion: Bacterial ecology appears not different from that usually observed in sickle-cell children. Salmonella and Escherichia coli were predominant.

关键词： Sickle Cell Disease Child Infection Niger