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1.
Qatar Med J ; 2023(2): 23, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38025328

RESUMO

Objective: Common variable immunodeficiency (CVID) is a complex inborn error of humoral immunity with complications of both infectious and non-infectious origins. Classifications of CVID patients provide a clearer understanding of the pathogenesis, prediction, and management of non-infectious complications. This study aims to classify Moroccan CVID patients based on the European classification (EUROclass). Materials and Methods: We recruited 20 CVID patients meeting standard diagnostic criteria (5-6). After collecting clinical and demographic data, we used flow cytometry to analyze B-cell subsets and group patients and assess the relation of each group with clinical manifestations. Results: 90% of the patients in our cohort study had a history of respiratory infections. The noninfectious manifestations included splenomegaly, autoimmunity, lymphadenopathy, and granulomatous diseases diagnosed in 50%, 45%, 40%, and 25% of patients, respectively. We observed significant co-occurrence of splenomegaly with autoimmunity and granulomatous diseases to a lesser extent. Patients had a significant reduction in total, switched memory, marginal zone-like, plasma blasts, and a substantial increase in the percentage of activated B cells, suggesting a defect in the late phases of B-cell differentiation. This condition was linked with an increased occurrence of splenomegaly and granulomatous affections. Besides, patients also had an expansion of CD21low B-cells, which was strongly associated with splenomegaly. Conclusion: The classification of the first Moroccan cohort of CVID patients showed agreement with previous results. It suggests the possibility of adopting this approach on a global scale for better diagnosis and follow-up of CVID patients.

2.
Cureus ; 16(3): e57279, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38690506

RESUMO

Kaposi's sarcoma (KS), linked to human herpesvirus 8 (HHV8), manifests in various clinical forms with iatrogenic KS uniquely tied to immune dysregulation induced by medical interventions. This study describes a 58-year-old male of sub-Saharan origin with a medical history of segmental and focal hyalinosis treated with methylprednisolone and mycophenolate mofetil. The patient developed skin lesions on both thighs, accompanied by post-prandial vomiting and abdominal pain. Clinical examination revealed flesh-colored nodules on the thighs and inguinal lymphadenopathy. Biopsy confirmed the diagnosis of KS, exhibiting positive nuclear labeling to anti-HHV8 and negative HIV serology. Additionally, radiological findings from the thoracic-abdominal-pelvic computed tomography (CT) scan significantly contribute to our understanding of the multiorgan involvement associated with KS in this case, providing valuable insights for diagnosis and therapeutic considerations. This case highlights the iatrogenic subtype of KS, linked to immunosuppression from prior medical interventions. Notably, gastrointestinal involvement was evident, with lesions in the stomach and small intestine. Intravenous paclitaxel administration resulted in a positive clinical response. This study underscores the importance of clinical vigilance, endoscopic evaluation, and early intervention in the nuanced diagnosis and management of iatrogenic KS.

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