Open AccessHemophagocytic lymphohistiocytosis presenting with acute liver failure and central nervous system involvement in early infancy
Author(s) -
Mukesh Kumar,
Nirupama Kothari,
Gupta Bd,
Neeraj Gupta
Publication year - 2018
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/ijpm.ijpm_264_17
Subject(s) - hemophagocytic lymphohistiocytosis , central nervous system , medicine , liver failure , pediatrics , pathology , disease
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal and likely underdiagnosed disease characterized by unregulated histiocyte proliferation, hypercytokinemia and hemophagocytosis, causing life-threatening tissue damage and organ failure. We report a case of a 56-day-old infant presenting with fever, acute liver failure, and neurological manifestations as presenting features that succumbed to rapidly progressive HLH. Our objective is to emphasize the importance of early diagnosis by high suspicion in varied initial presentation of HLH so that life-saving therapy may be instituted in time.