Hemophagocytic syndrome in malaria and kala-azar.

ABSTRACT

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.