ABSTRACT
Haemophagocytic syndrome (HPS) secondary to infections occurs due to excessive, non-malignant proliferation of histiocytes, with resultant haemophagocytosis. The syndrome is essentially treatable, provided timely etiological diagnosis is achieved. In this report, we present a rare case of a child who hailed from Uttaranchal and presented with severe hepatitis. Bone marrow examination revealed an unexpected diagnosis of HPS secondary to visceral leishmaniasis. Despite initiating appropriate antileishmanial treatment, the child had a fatal outcome.
Subject(s)
Antiprotozoal Agents/therapeutic use , Bone Marrow/parasitology , Child, Preschool , Fatal Outcome , Hepatitis/parasitology , Humans , Leishmaniasis, Visceral/complications , Lymphohistiocytosis, Hemophagocytic/parasitologyABSTRACT
Hemophagocytic syndrome is a clinicopathological entity characterized by systemic proliferation of cells of the monocyte-macrophage-histiocytic lineage associated with fever, cytopenias, hepatosplenomegaly, lymphadenopathy and coagulopathy. Two forms of the syndrome have been described-familial hemophagocytic lymphohistiocytosis (FHL) of infants and reactive hemophagocytosis syndrome (RHS) encountered at any age. The underlying diseases are heterogenous which include, systemic lupus erythematosus and infections- bacterial, viral, fungal and parasitic. Mechanisms of RHS remain unclear, but cytokines may play a role. Risk factors associated with death are worsening anemia, thrombocytopenia during treatment, presence of DIC, and increase in serum ferritin and â-2 microglobulin and jaundice. We discuss the peripheral blood and bone marrow findings in 14 cases RHS with associated Kala-Azar (9 cases) and malaria (plasmodium vivax-2, plasmodium falciparum-3 cases). Three patients of Kala-azar expired- two due to hemorrhagic shock and one as a result of antimony related myocarditis.