Familial haemophagocytic lymphohistiocytosis: rare cause of acute liver failure in a neonate--a case report

Received: November 12, 2012
Accepted: May 13, 2013
Ref: Ojha R, Zia S, Al-Kasim F, Al-Hussaini AR. Familial haemophagocytic lymphohistiocytosis: rare cause of acute liver failure in a neonate--a case report. Internet J Med Update. 2014;9(1):50-53.

Rahul Ojha* FRACP, Sayed Zia** MRCP, Fauwaz Al-Kasim** MRCP and Abdul Rehman Al-Hussaini A** FRACP

*Sydney Medical School Nepean, The University of Sydney, NSW, Australia
**Children’s Hospital, Riyadh Medical Complex, Riyadh, Kingdom of Saudi Arabia

(Corresponding Author: Dr. Rahul Ojha, Department of General Paediatrics, Nepean Hospital. Lecturer, Sydney Medical School Nepean, The University of Sydney, NSW, Australia; Cell: +61-2-47342134; Email: ojharahul@yahoo.co.in)

ABSTRACT

Familial haemophagocytic lymphohistiocytosis (FHLH) is a rare life-threatening disorder of the immune system characterized by proliferation and infiltration of hyperactivated macrophages and T-lymphocytes and manifesting as acute illness with prolonged fever, cytopenias, and hepatosplenomegaly. We describe a one-month-old female with FHLH and rapidly progressing liver failure. To conclude, FHLH should be included in the differential diagnosis of acute liver failure in neonates and young infants.

KEY WORDS: Familial haemophagocytic lymphohistiocytosis; Acute liver failure; Neonate; Case report

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