Case Report Haemophagocytic lymphohistiocytosis: a cause of unresponsive malaria in a 5-year-old girl Manish Bhagat, Sujata Kanhere, Purvi Kadakia, Varsha Phadke, Riya George, Kushagra Chaudhari Department of Paediatrics, K. J. Somaiya Medical College and Hospital, Mumbai, India A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytope- nia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglycer- idaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone. Keywords: HLH, Malaria, Megaloblastic anaemia Introduction Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory syndrome char- acterised by excessive activation and proliferation of T-cells and cells of macrophage-histiocytic lineage. HLH has been described with severe infections, malignancies, autoimmune conditions and drug hyp- erensitivity reactions. 1,2 Malaria-related HLH has been uncommonly reported. 3–8 We report a rare case of mixed malaria (Plasmodium falciparum and Plasmodium vivax) complicated by HLH and mega- loblastic anaemia in a previously healthy girl. Case Report A 5-year-old girl was admitted to K. J. Somaiya Medical College and Hospital, Mumbai with a 4-day history of fever, vomiting for 2 days and abdominal pain on the day of admission. The fever was high grade associated with chills and rigors. Vomiting was non bilious and non-projectile. Abdominal pain was periumbilical and dull aching. There was no history of cough, dysuria, oliguria, headache or altered behaviour. On examination, she was afebrile; pulse rate was 96/min, respiratory rate 25/min and blood pressure 90/60 mm Hg. She was pale and icteric without petechiae or pedal oedema. Systemic exam- ination demonstrated peri-umbilical tenderness, asc- ites and hepatosplenomegaly (liver and spleen each 2 cm below costal margins). Investigations. Haemoglobin (Hb) was 6 g/dl, total leucocyte count (TLC) 23.2610 9 /L (neutrophils 38%, lymphocytes 60%), platelet counts 236610 9 /L and erythrocyte sedimentation rate (ESR) 2105 mm in the first hour. Peripheral blood smear demonstrated trophozoites of Plasmodium vivax and gametocytes of Plasmodium falciparum. Total serum bilirubin (TSB) was 14.7 mmol/L (5–17) and creatinine was 97.2 mmol/L (27–62). The patient was treated with intravenous artesunate, clindamycin and cefotaxime. On the second day, she had haematuria, severe pallor and deep icterus. Repeat haemogram showed: Hb 4.4 g/dl, TLC 23.2610 9 /L, platelet count 30610 9 /L and reticulocyte count 1%. Urine analysis showed pus cells 3–4/high power field (hpf), red blood cells 5–6/hpf and proteins 1z; no casts were seen. TSB was 39.3 mmol/L. She was transfused with packed red blood cells (PRBC) and platelets. Glucose 6 phosphate dehydrogenase (G6PD) was normal. Post transfusion, Hb was 5.8 g/dl, TLC 5.5610 9 /L and platelet count 66610 9 /L. Meanwhile, the child continued to have intermittent fever (Tmax 38uC). The temperature Correspondence to: S Kanhere, K. J. Somaiya Medical College and Hospital, Mumbai, India. Fax: z91 22 2 409 1855; email: drsujatakanhere@ yahoo.co.in ß W. S. Maney & Son Ltd 2014 DOI 10.1179/2046905514Y.0000000163 Paediatrics and International Child Health 2014 VOL. 000 NO. 000 1