Adult Hemophagocytic Lymphohistiocytosis in Sub-Saharan Area: A Retrospective Study of 26 cases.
adult
hemophagocytic lymphohistiocytosis
tropical environment
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
13 Mar 2020
13 Mar 2020
Historique:
entrez:
21
3
2020
pubmed:
21
3
2020
medline:
21
3
2020
Statut:
epublish
Résumé
Introduction and background Hemophagocytic lymphohistiocytosis (HLH) is a condition caused by inappropriate stimulation of macrophage cells with hemophagocytosis. This paper aims to describe its diagnostic specifics and etiology and seeks to identify the factors that affect its prognosis in the black African adult population. Methods A retrospective multicentre study was carried out in three medical units in Senegal: Department of Internal Medicine at Pikine Teaching Hospital, and Department of Internal Medicine and Department of Nephrology at Aristide Le Dantec Teaching Hospital; the study covered the period from January 1, 2012 to March 30, 2015. This study included patients aged 18 years and older with a Hemophagocytosis Score (HScore) of ≥202 (with probabilities of acquired HLH of >90%). The data was obtained through medical records. Results In total, 26 patient files were included. The average age of the patients was 41 years, with a male-to-female ratio of 2.25:1. Fever and cytopenia were frequent. Other clinical signs included peripheral lymphadenopathy (69.2%), hepatomegaly (53.8%), splenomegaly (34.6%), neurological disorders (34.5%), and respiratory disorders (15.3%). Thrombocytosis was noted in three cases. Renal involvement was present in eight patients, with one case of collapsing glomerulopathy. The bone marrow aspirate revealed myelodysplasia in 12 patients. The dominant etiologies of HLH were hematological malignancies and infections. The mortality rate of HLH was 73%. Male gender and non-etiological targeted therapy were significantly associated with mortality. However, the age of <40 years in patients and current systemic disease in some cases were correlated with survival. The use of etoposide had no significant impact on the prognosis of our patients. Conclusion A high rate of male predominance, important central nervous system disorders, myelodysplasia, and paradoxical thrombocytosis were found to be the distinct features of adult HLH in our study population.
Identifiants
pubmed: 32195069
doi: 10.7759/cureus.7258
pmc: PMC7075479
doi:
Types de publication
Journal Article
Langues
eng
Pagination
e7258Informations de copyright
Copyright © 2020, Diack et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
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