Abstract Number: PB1044
Meeting: ISTH 2021 Congress
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially lethal condition. It can be primary or secondary and can complicate other conditions: neoplastic, infectious or autoimmune. Systemic diseases are an uncommon cause of HLH.
Aims: The aim of this work is to study the clinico-biological and etiological characteristics of HLH cases during systemic diseases.
Methods: Retrospective study carried out over a period of 22 years including patients presenting a (LHL) whose diagnosis was retained according to the Histiocyte Society 2004 criteria. Patients presenting a (LHL) related to a systemic disease were studied.
Results: Five patients out of a total of 10 patients with HLH (50%) were female with an average age of 39.4years. The reason for hospitalisation was febrile pancytopenia in 60% of cases, febrile polyarthralgia in 10% of cases and febrile dyspnoea in 10% of cases. An alteration in general condition was found in 4 patients, fever in all patients, hepatomegaly in 1, splenomegaly in 3, adenopathy in 1 patient and erythematous skin rash in 2 patients. Biology revealed a biological inflammatory syndrome in 60%cases, cytopenia in 80%patients: normocytic normochromic anaemia in 80% cases of inflammatory type in 40% cases and autoimmune in 40% cases, leukopenia in 60% cases, lymphopenia in all cases and thrombocytopenia in 80% of patients. Hyperferritinemia was objectified in all patients, hypertriglyceridemia in 4 patients and hepatic cytolysis in 3 patients. The myelogram revealed multiple images of haemophagocytosis in 80% cases. The LHL was secondary to: a severe relapsing SLE in 4 cases and Still’s disease in 1 case. The treatment was both symptomatic and etiological using high dose corticosteroid therapy in all case. The evolution was favourable for all our patients.
Conclusions: Systemic diseases are among the aetiologies of (LHL) whose diagnosis can be difficult because of the presence of several clinical and biological signs common to both pathologies.
To cite this abstract in AMA style:Chabchoub I, Ben Salah R, Abida F, Frikha F, Chaari M, Bahloul Z. Hemophagocytic Lymphohistiocytosis Complicating Systemic Diseases [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/hemophagocytic-lymphohistiocytosis-complicating-systemic-diseases/. Accessed December 7, 2021.
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