Raja Bhattacharya

Urmimala Bhattacharjee

Ritasman Baisya

Pallab Biswas

Akashdip Bhattacharya

Reet Banerjee
Hemophagocyticlymphohistiocytosis, Autoimmune disease, Infection. ,

Hemophagocyticlymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition due to an uncontrolled and ineffective immune response. Cardinal symptoms include prolonged fever, lymphadenopathy, hepatosplenomegaly, cytopenias and hemophagocytosis on biopsy. Biochemical examination reveals elevated serum ferritin, triglycerides and low fibrinogen. It is traditionally classified into primary or genetic form and secondary or acquired form. The secondary form complicates different underlying infection, malignancy, auto-immune and auto-inflammatory conditions. A high degree of clinical suspicion is necessary for prompt diagnosis and early initiation of treatment, which is crucial to decrease the significant mortality associated with this clinical entity. In this article we have retrospectively analysed the clinical data of all secondary HLH cases admitted in general medicine ward of our hospital over a period of one year. This article aims to review the varied clinical presentation, prognosis and treatment of HLH.

8 , 1 , 2018