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Connought Place, New Delhi, India ... cases in India are due to Leishmania donovani.1 The disease ... transferase-221 IU/L, Alkaline phosphatase-1865 IU/L.
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Clinical Brief

Hemophagocytic Syndrome Associated with Visceral Leishmaniasis Shilpi Agarwal, Shashi Narayan, Sunita Sharma, Eram Kahkashan and A.K. Patwari1 Department of Pathology and 1Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children's Hospital Connought Place, New Delhi, India

ABSTRACT. The present paper reports a case of 6-year-old male child, suffering from pallor, fever and hepatosplenomegaly. A clinical diagnosis of enteric fever with a second possibility of malaria was considered. Laboratory findings included bicytopenia, hyperbilirubinemia and raised liver enzymes. Bone marrow examination revealed active hemophagocytosis. On extensive search few amastigote forms of Leishmania donovani were seen. Patient was negative for other viral, bacterial and malaria infections. The final diagnosis of hemophagocytic syndrome associated with visceral leishmaniasis was made. There was response of anti-Leishmanial treatment with improvement in clinical condition. [Indian J Pediatr 2006; 73 (5) : 445-446] Key words : Hemophagocytic syndrome; Visceral leishmaniasis

Kala-azar (Visceral leishmaniasis) is caused by Leishmania sp. Out of 22 known species, the majority of cases in India are due to Leishmania donovani.1 The disease is endemic in north eastern India. Cutaneous, mucocutaneous and visceral leishmaniasis are common manifestations. Hemophagocytic syndromes are macrophage related histiocytic disorders which are significant in terms of morbidity and mortality. Primary hemophagocytic lymphohistiocytosis (HLH), the prototypical hemophagocytic syndrome is either familial, occurring in obvious familial setting or as a sporadic event.2 The more common form is seen during infancy and childhood, and is inherited as an autosomal recessive trait. Possible loci for a responsible gene/genes have recently been located on long arms of chromosomes 9 & 10.3,4 The disease is invariably fatal with a median survival of two months without therapy. Secondary HLH has been associated with infections, parasitic infestations, malignant disorders, genetic disorders such as Chediak-Higashi disease, Griscelli disease, XLP syndrome, conditions like lipid rich parenteral alimentation and rheumatoid arthritis.2 Hemophagocytosis as a presentation of visceral leishmaniasis is a very rare event and can cause considerable diagnostic difficulty. CASE REPORT A 6-year-old male child, resident of Bihar was admitted to

Correspondence and Reprint requests : Dr. Sunita Sharma (Professor) Department of Pathology, Lady Hardinge Medical College, Shaheed Bhagat Singh Margh, Connought Place, New Delhi-110001

Indian Journal of Pediatrics, Volume 73—May, 2006

Kalawati Saran Children Hospital with complaints of fever, pallor and loss of appetite for 15 days and yellow discoloration of eyes for 3 days. The child had achieved all developmental milestones at an appropriate age and had been immunized for common diseases. On general examination, the patient was asthenic, had pallor and mild icterus. Systemic examination revealed hepatosplenomegaly of 5 cm and 3 cm below costal margin respectively. Cardiovascular system, respiratory system and central nervous system were within normal limits. A clinical diagnosis of enteric fever with a second possibility of malaria was considered. The child was given cephalosporin and antimalarials but did not respond to therapy. Laboratory investigations revealed hemoglobin 7.8 g/ dl, TLC 12.0 × 103/µl, platelets 17.0 × 103/µl, MCV 71.7 fl, MCH 20.8 pg, MCHC 29.0 g/dl and reticulocyte count 0.2%. Wright stained peripheral smear showed microcytic hypochromic red cells and was negative for malaria parasite. Liver functions were deranged with serum bilirubin3.2mg/dl (direct2.1mg/dl, indirect1.1mg/dl), Aspartate amino transferase 441 IU/L, Alanine amino transferase-221 IU/L, Alkaline phosphatase-1865 IU/L. Serological markers i.e. anti HCV, anti HAV and HBs Ag were negative. Renal function tests were within normal range. Serum Widal was negative. Wright stained bone marrow smears showed hyper cellular marrow with normoblastic erythroid hyperplasia, M:E 1:1. Myeloid and megakaryocytic series were normal in maturatiion and morphology. There was a marked increase in histiocytes with a prominent hemophagocytosis. On extensive search few amastigote forms of L. donovani were seen both intracellularly and extracellularly (Fig. 1). Immunofluorescence test for anti Leishmanial antibody was positive in (1:200) titre. Serology for TORCH, HIV 445

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Shilpi Agarwal et al These findings were concordant with the diagnostic criteria defined by HLH study group of the Histiocyte Society in 1991. 2 A vigorous hunt for the cause of hemophagocytosis showed that the patient was negative for viral markers (herpes virus, cytomegalovirus, hepatotropic viruses and rubella virus), bacterial infections, toxoplasmosis and malaria. However on extensive search few amastigotes froms of Leishmania donovani could be domonstrated in the bone marrow aspirate. The patient responded to anti Leishmanial treatment with regression of hepatosplenomegaly, fever and improvement of blood parameters. Fig 1. Hemophagocytosis and extracellular amastigotes of Leishmania donovani (Wright stain 1000x)

and RAfactor was negative. A final diagnosis of visceral leishmaniasis with secondary hemophagocytic syndrome was made. Patient was given sodium stibogluconate (20 mg/kd body weight) for 20 days after which a repeat bone marrow aspiration was negative for leishmania donovani. DISCUSSION There are derangements of several metabolic and hematological parameters in hemophagocytic syndrome. The diagnostic criteria is given by Henter et al.4 includes (a) Clinical criteria of fever and splenomegaly. (b) Laboratory criteria: cytopenias (affecting two of three lineages in the peripheral blood), haemoglobin (