Hemophagocytic Lymphohistiocytosis: About 8 Cases
Journal Title: Saudi Journal of Medical and Pharmaceutical Sciences - Year 2018, Vol 4, Issue 3
Abstract
Abstract:Hemophagocytic lymphohistiocytosis (HLH) is a clinical-biological entity characterized by a hyperinflammatory state following the deregulation of the cytotoxic immune response, causing histiocytic proliferation with significant hemophagocytic activity in the bone marrow as well as the massive release of inflammatory cytokines. The diagnosis is established by the association of nonspecific clinical and biological signs. The aim of this study was to describe the clinical and laboratory presentation to determine the underlying pathologies and clarify the evolution and the prognosis of adult patients with HLH in our healthcare setting. A retrospective study of patients with HLH was conducted in the Military Teaching Hospital, Rabat, between January 2015 and January 2017. We were able to identify eight patients. Six were men, and the average age at diagnosis was 58 years. Infectious agents were responsible in 3 cases, malignant lymphoma in 3 others, one case of systemic lupus erythematosus, and in the last case etiology remains unknown. Clinical characteristics such as fever (100%), splenomegaly (62,5%), and lymphadenopathy (37,5%) were observed. Laboratory values presented with panytopenia (62, 5%), thrombopenia and anemia (100%), increased ferritin (100%), lactate dehydrogenase (87, 5%), triglycerides (62, 5%), and decreased fibrinogen (50%). Bone marrow showed hemophagocytis in all cases. The prognosis was poor. Five patients died. Absence of specific signs makes early diagnosis difficult. The prognosis varies according to the series but remains very unfavorable. Studies are needed to assess prevalence and develop a severity score and therapeutic recommendations to improve HLH prognosis. Keywords:Hemophagocytic lymphohistiocytosis, Infections, Macrophage.
Authors and Affiliations
Souhail Mouline
Keywords
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