Journal of Pediatric Infectious Diseases 6 (2011) 269–271 269 DOI 10.3233/JPI-2012-0334 IOS Press Rapidly fatal reactive hemophagocytosis syndrome associated with visceral leishmaniasis Lokesh Kumar Tiwari a , Devidayal a,* , Reena Das b and Sudhanshu Grover a a Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India b Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, India Received 17 May 2011 Revised 20 July 2011 Accepted 26 July 2011 Abstract. Hemophagocytosis syndrome secondary to visceral leishmaniasis (VL) is rare. The diagnosis is likely to be missed because of its clinical presentation similar to VL. Because Leishmaniasis is endemic in many states of India, primary treating physicians needs to be sensitized about the existence of this potentially fatal but treatable entity. Further, it is important to differentiate infection associated reactive hemophagocytosis syndrome (RHS) from primary hemophagocytosis to avoid unnecessary and potentially harmful cytotoxic therapy in infection associated hemophagocytosis. In this report, a young boy who developed accelerated RHS secondary to VL is presented. He succumbed to acute haematological complications of RHS while undergoing procedures for establishing the diagnosis of his primary illness. Keywords: Hemophagocytosis syndrome, visceral leishmaniasis, children 1. Introduction Hemophagocytic lymphohistiocytosis (HLH), also known as Hemophagocytic syndrome (HS) is charac- terized by activation and uncontrolled non-malignant proliferation of T lymphocytes and macrophages, lead- ing to cytokine overproduction resulting into acute febrile illness, hepatosplenomegaly, pancytopenia, marked hypofibrinogenemia, and hypertriglyceride- mia [1]. Primary HLH is familial, and is inherited as an autosomal recessive trait. The disease is invariably fatal with a median survival of 2 months without ther- * Correspondence: Dr. Devidayal, Department of Pediatrics, Ad- vanced Pediatrics Center, Postgraduate Institute of Medical Edu- cation and Research, 160012, Chandigarh, India. Tel.: +91 172 2755657, +91 172 2742017; Fax: +91 172 2744401, 2745078; E- mail: drdevidayal@indiatimes.com. apy. Secondary HLH has been associated with viral, bacterial, fungal, and parasitic infections, a broad spec- trum of malignancies and genetic disorders, such as Che’diak-Higashi disease, Griscelli disease, XLP syn- drome, and familial erythrophagocytic lymphohistio- cytosis [2]. The association of visceral leishmaniasis (VL) with hemophagocytosis has previously been re- ported but is poorly documented in children [3–5]. We report a child presenting with features of HLH sec- ondary to VL. 2. Case report A seven years old boy, resident of Bihar, Central In- dia was admitted with complaints of intermittent, mod- erate grade fever for 3 months and occasional abdom- inal discomfort for last 1 month. In the 10 days pri- 1305-7707/11/$27.50  2011 – IOS Press and the authors. All rights reserved AUTHOR COPY