Hemophagocytic Lymphohistiocytosis in Adults and Adolescents - Experience from a Tertiary Care Centre in South India

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of immune dysregulation. It is characterized by hypercytokinemia and macrophage activation resulting in fever, cytopenia, splenomegaly and hyperferritinemia leading to fatal outcomes if untreated. Aim: To study clinical profile, diagnostic and etiological workup, treatment and prognosis of hemophagocytic lymphohistiocytosis patients. Materials and Methods: We report retrospective analysis of 8 cases of adult and adolescent HLH admitted over a period of 2 years at JIPMER, a tertiary care centre in South India. Results: Mean age of patients was 27 year (range 13 to 57 years) and 3 were adolescents. Median duration of symptoms was 10 days (5-60 days). Common presenting symptoms were fever, jaundice, abdominal pain, rash and seizures. Physical findings included pallor, icterus, splenomegaly, hepatomegaly and lymphadenopathy. Laboratory findings were variable cytopenia with pancytopenia in 65% cases, hyperferritinemia (100%), hypertriglyceridimia (75%) and elevated serum bilirubin (62.5%) and liver enzymes (87.5%). Underlying cause could be detected in only 6 patients with one each of dengue fever, lymphoma, tuberculosis, EBV infection, scrub typhus and juvenile idiopathic arthritis. Median overall survival at the end of 1.5 years was 62%. Conclusion: Low threshold for suspicion, adequate evaluation and timely treatment can improve outcome in patients of HLH.

Keywords

• immune dysregulation
• epstein- barr virus
• nk/t cell lymphoma