Fatal hemophagocytic lymphohistiocytosis, probably secondary to leishmaniasis, in an immunocompetent adult, with no response to any treatment

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a rare syndrome, attributed to genetic and acquired factors and is associated with overactivation of macrophages. There are better chances of treatment, when HLH is triggered by an infection. Herein, we describe a rare case which raises questions about this statement. A 53 year’s old, immunocompetent man, presented with relapsing fever for four months. The patient underwent a full workup with no diagnostic findings, apart from detectable IgG antibodies in the plasma and PCR positive for Leishmania in bone marrow aspirates. The patient received liposomal amphotericin B with no fever remission. Patient’s blood tests revealed thrombocytopenia, anemia, high ferritin levels and hypertriglyceridemia. Image analysis revealed splenomegaly. Diagnostic criteria of hemophagocytic syndrome were fulfilled. Genetic testing was negative. The patient received two additional cycles of liposomal amphotericin B, with no response. Finally, he received etoposide, cyclosporine and dexamethasone, according to 2004 HLH protocol. After a long hospitalization the patient passed away.