Hemophagocytic Lymphohistiocytosis Associated With Visceral Leishmaniasis

Abstract

A 2-year-old child presented with fever and hepatosplenomegaly. Laboratory findings showed pancytopenia, hypertriglyceridemia, hyperferritinemia, and high levels of soluble-IL2 receptors. Initial bone marrow aspiration and biopsy revealed mild hemophagocytosis. A diagnosis of hemophagocytic lymphohistiocytosis was made and appropriate treatment was begun. Repeated marrow aspiration performed because of lack of clinical response revealed Leishmania amastigotes in macrophages in addition to active hemophagocytosis. Treatment with liposomal amphotericin resulted with rapid recovery. Visceral leishmaniasis should be considered in the differential diagnosis of hemophagocytic syndrome.

Hemophagocytic Lymphohistiocytosis Associated with Visceral Leishmaniasis in Late Adulthood

“…1 Earlier reports have shown that immunosuppressive therapy usually increases the number of amastigotes present on a bone marrow smear. [6][7][8] Despite the fact that our patient had completed a 15-day course of steroids, we detected very few amastigotes in his bone marrow. Indeed, previous reports have shown that the first bone marrow aspirate often fails to establish the presence of Leishman-Donovan bodies in 36.3% of cases.…”

The Hemophagocytic Syndrome in an Immunocompromised Patient: A Diagnostic Challenge

“…The diagnosis in the presence of hemophagocytosis is elusive and usually delayed, with one study showing delays of up to 134 days (5). In addition, the absence of parasite visualization may lead to an erroneous diagnosis of one of the hematological neoplasms which display hemophagocytosis, resulting in aggressive chemotherapy treatments and/or bonemarrow allografting, as some authors have reported (1,2,5,6,8,9).…”

“…Specific serology is useful in immunocompetent patients, but seroconversion can be delayed or nonexistent in immunocompromised individuals (5). Polyclonal B-cell activation by this parasite produces hypergammaglobulinemia and high titers of antileishmania antibodies, except when hemophagocytosis develops (5,6). The hemophagocytic syndrome seldom occurs in VL but, if present, parasites become very scarce in bone marrow smears for unexplained reasons and, thus, are difficult to detect (7,8).…”

Fatal haemophagocytic syndrome and hepatitis associated with visceral leishmaniasis

“…[1][2][3][4][5][6][7][8][9] In a review of all the published cases of HPS associated with VL in English literature, we found that almost all of them were cured following treatment. In the present case, there was a prolonged delay in the initiation of treatment due to its failure to be diagnosed as a case of VL at his native place.…”

“…Haemophagocytic syndrome (HPS) is a potentially fatal condition characterized by excessive proliferation and activation of histiocytes with resultant phagocytosis of blood elements (haemophagocytosis). 1,2 There is an overlap in the clinical presentations of VL and HPS, both of which present with fever, hepatosplenomegaly and pancytopenia. However, VL patients consistently have marked hypergammaglobulinaemia and the patients of HPS have haemophagocytes in the marrow with or without coagulopathies.…”