Haemophagocytic syndrome associated with visceral leishmaniasis
Abstract number: 902_p625
Haemophagocytic syndrome, more properly referred to as haemophagocytic lymphohistiocytosis (HLH), is a rare distinct clinical entity of infancy and early childhood. HLH is commonly associated with various systemic infections and to a broad spectrum of malignancies and genetic disorders. Although the association of HLH and visceral leishmaniasis (VL) has been rarely described, the diagnosis of the disease may be difficult when VL and HLH together present in adults. We report a case of haemophagocytosis in a 34-year-old man to draw attention to the difficulties in diagnosing VL when accompanied by HLH.
A 34-year-old man presented with a 4-week history of fever, malaise, poor feeding and abdominal pain. Past medical history was unremarkable. Physical examination revealed high fever (39.2°C) with pallor, hepatomegaly and splenomegaly. Lymph nodes were not enlarged and vital signs were normal. The laboratory findings showed pancytopenia and elevated erythrocyte sedimentation rate; serum triglycerides were increased to 267 mg/dL with normal cholesterol. Transaminase activity and total bilirubin were high, whereas serum fibrinogen was low with elevated circulating fibrin degradation products. There was no serological evidence for infection with cytomegalovirus, EpsteinBarr virus and toxoplasmosis. Blood cultures were negative. The patient symptoms deteriorated within 24 h and he was referred to intensive care unit where intestinal bleeding with disseminated intravascular coagulation occurred. The patient died 48 h after admission in the ICU. Bone marrow and liver biopsy performed after death were compatible with diagnosis of HLH, whereas peripheral blood culture for parasites yielded Leishmania infantum, identified as zymodeme MON1 which is predominant in our country.
Leishmaniasis should be considered when discussing the cause of haemophagocytosis in countries where the disease is endemic, such as Tunisia. When revealed by haemophagocytosis, diagnosis may be difficult particularly in adults."
|Session name:||XXIst ISTH Congress|
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