Hemophagocytosis is although a common phenomenon seen in bone marrow but

Hemophagocytosis is although a common phenomenon seen in bone marrow but at times it is overlooked. count of 0.84 109/L, hemoglobin of 69.6 g/L, total reddish blood cell count of 2.3 1012/L and platelet count of 67 109/L. Peripheral blood smear examination revealed predominantly normocytic normochromic erythrocytes with leucopenia and marked neutropenia. The serum aspartate aminotransferase level (50 IU/L) and serum alkaline phosphatase was elevated Adamts4 (234 IU/L) with hyponatremia (131.7 mmol/L). Serum ferritin (750 g/L) and triglycerides levels (350 mg/dl) were also increased with hypoalbuminemia (1.4 g/dl). The patient was nonreactive for hepatitis B surface antigen and hepatitis C virus antibody. In view of pancytopenia, the patient was subjected to bone marrow examination. Jenner Giemsa stain of bone marrow aspirate smears showed predominantly normoblastic maturation with adequate and functional megakaryocytes. The smears also revealed (LD) amastigote forms (LD bodies) along with the phenomenon of hemophagocytosis demonstrating reactive histiocytes containing phagocytosed LD bodies along with phagocytosed leukocytes [Physique 1]. Based on these findings the case was diagnosed as leishmaniasis with HLH syndrome. The patient responded adequately to amphotericin and was later discharged. Open in a buy PSI-7977 separate window Figure 1 Bone marrow aspirate showing phenomenon of hemophagocytosis demonstrating reactive histiocytes containing phagocytosed leukocyte and bodies (Jenner Giemsa; 1000) Hemophagocytic lymphohistiocytosis is described as highly stimulated and ineffective immune response that may be familial or acquired and is considered as life-threatening condition.[3] Hypersecretion of pro-inflammatory cytokines such as interferon gamma, tumor necrosis factor alpha, and CD8 T-cells are said to play a crucial role in the pathogenesis of HLH.[3,4] buy PSI-7977 HLH associated with leishmaniasis is usually rarely reported in the literature and at times the diagnosis of this association may be challenging.[5] It is necessary to distinguish between leishmaniasis showing hemophagocytosis on bone marrow and a case of secondary HLH with leishmaniasis. Bone marrow examination, which may be at times repeated along with relevant biochemical and laboratory examination, is essential for a definite diagnosis. The early recognition of HLH with leishmaniasis followed by prompt treatment is necessary to avoid poor prognosis of such cases. REFERENCES 1. Bode SF, Lehmberg K, Maul-Pavicic A, Vraetz T, Janka G, Stadt UZ, et al. Recent improvements in the diagnosis and treatment of hemophagocytic lymphohistiocytosis. Arthritis Res Ther. 2012;14:213. [PMC free article] [PubMed] [Google Scholar] 2. Henter JI, Horne A, Aric M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. buy PSI-7977 2007;48:124C31. [PubMed] [Google Scholar] 3. Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: Pathogenesis and treatment. Hematology Am Soc Hematol Educ Program 2013. 2013:605C11. [PubMed] [Google Scholar] 4. Jordan MB, Hildeman D, Kappler J, Marrack P. An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+T cells and interferon gamma are essential for the disorder. Blood. 2004;104:735C43. [PubMed] [Google Scholar] 5. Bode SF, Bogdan C, Beutel K, Behnisch W, Greiner J, Henning S, et al. Hemophagocytic lymphohistiocytosis in imported pediatric visceral buy PSI-7977 leishmaniasis in a nonendemic area. J Pediatr. 2014;165:147C53.e1. [PubMed] [Google Scholar].