Hemophagocytic lymphohistiocytosis: epidemiological, clinical and biological profile

Abstract

Background/aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical, biological, and pathological entity that is rare but has certainmorbidity that may be life-threatening. This work aims to establish a focus on the hemophagocytic lymphohistiocytosis and analyzedifferent aspects of diagnosis while emphasizing the biological data.Materials and methods: We report the results of a retrospective study conducted in the hematology department of Avicenna Hospital inMarrakesh. Thirty-one patients with hemophagocytic lymphohistiocytosis were enrolled.Results: The clinical presentation was dominated by fever and deterioration of the general state for almost all our patients. Splenomegalywas objectified in 90% of the patients. Hepatomegaly, lymphadenopathy, and hemorrhagic manifestations were observed in almost50% of the patients. Biological assessments revealed bi- or pancytopenia in 96% of the patients, and coagulation disorders in 51% ofthe patients. On the other hand, hyperferritinemia was found in 84% of the patients, and hepatic cytolysis and hypertriglyceridemia inhalf of the patients. Hemophagocytosis was observed in all bone marrow samples taken from our patients. Concerning the evolution ofpatients, in 38.5% of the patients, the evolution was favorable with regression of clinical and biological signs. Twenty six percent of thepatients had died, mainly from multiple organ failure and disseminated intravascular coagulation.Conclusion: HLH is a diverse condition with many causes and is likely to be under-recognized, which contributes to its high morbidityand mortality. Clinicians need to be able to recognize the signs and symptoms commonly seen in HLH and actively pursue this diagnosisin the cases of undiagnosed febrile illness with multiorgan dysfunction. Early recognition is crucial for any reasonable attempt at curativetherapy to be made.