Purpose: To study the etiology & outcome of patients with hemophagocytic lymphohistiocytosis (HLH) admitted in the medical male & female wards of Govt. Medical College, Kozhikode, Kerala. Methods: We report 27 cases of HLH in patients > 13 years admitted over a period of 1.5 years at Govt. Medical College, Kozhikode, a tertiary care centre in South India. Results: The most common age group was 25-50 years. 21 were males. Mean duration of symptoms was 11 days (7-21 days). Common presenting symptoms were fever, anorexia, jaundice, breathlessness and altered sensorium. Common physical findings were pallor, icterus, splenomegaly, hepatomegaly, tachycardia and tachypnea. Laboratory findings were variable cytopenia with pancytopenia in 77.8% cases, hyperferritenimia (100%), hypertriglyceridimia (100%) and elevated liver enzymes (70.3%). Underlying cause could be detected in 17 patients with infectious etiolgy in 10, autoimmune in 5 & malignancy in 2. 15 patients (56%) expired at 1 week of treatment initiation. Mortality was high in patients with unidentified etiology. Conclusions: Infection was the most common cause followed by auto immune in this study. A low threshold for suspicion, proper evaluation for any secondary cause and early treatment can improve outcome in patients with HLH.
