Hemophagocytic Lymphohistiocytosis in Adults and Adolescents - Experience from a Tertiary Care Centre in South India
Journal Title: National Journal of Laboratory Medicine - Year 2017, Vol 6, Issue 1
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 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. Laboatory findings were variable cytopenia with pancytopenia in 65% cases, hyperferritenimia (100%), hypertriglyceridimia (75%) and elevated serum bilirubin (62.5%) and liver enzymes (87.5%). Underlynig 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.
Authors and Affiliations
Shailendra Prasad Verma, Rakesh Naik V, Debdatta Basu, Kolar Vishwanath Vinod, Rakhee Kar, Tarun Kumar Dutta
Keywords
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- EP ID EP375735
- DOI 10.7860/NJLM/2017/23907:2184
- Views 111
- Downloads 0
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