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New Indian Journal of Surgery

Volume  3, Issue 3, July - September 2012, Pages 254-254



Hemophagocytic Lymphohistiocytosis: A Life Threatening Rare Entity
Najmal Nazeer, V. Bafna, V. Kalrao
Bharati Vidyapeeth Deemed University Medical College, Pune, India
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 Hemophagocytic Lymphohistiocytosis (HLH) is a rare disorderofthe immune system,affecting macrophages that grow abnormally and accumulate in body organs which include liver, spleen, bone marrow, CNS and skin. There are 2 types of HLH: 

Primary: An inherited condition where there is a genetic mutation in:
• Perforin (PRF-1)
• Gene encoding Munc 13-4 protein which alters the NK & Tcell function.
Secondary: Triggered by infections (viral, bacterial, fungal), autoimmune disorders, primary immune deficiencies or cancer.

Corresponding Author : Najmal Nazeer