A rare case of secondary hemophagocytic lymphohistiocytosis with severe acute malnutrition in an infant

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological entity in children that presents with unremitting fever, organomegaly, cytopenias, hemophagocytosis by activated macrophages, and results in a fatal outcome in untreated cases. Other biochemical abnormalities include hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, increased soluble interleukin-2 receptor levels, histological evidence of benign hemophagocytic macrophage, and impaired natural killer cell activity. It is the result of an aberrant hyper inflammatory response that usually does not respond to conventional treatment. Secondary HLH is mostly associated with infection, malignancy, and autoimmune diseases and usually resolves on the treatment of the primary condition. Severe acute malnutrition (SAM) causing secondary HLH is rare, although hemophagocytosis in bone marrow without other diagnostic features of HLH is seen in many deficiency states including SAM. Here, we present a 1-year-old female child with SAM and secondary HLH focusing on clinical manifestation, diagnosis and treatment.