Table of Contents

HK J Paediatr (New Series)
Vol 12. No. 3, 2007

HK J Paediatr (New Series) 2007;12:205-211

Case Report

Haemophagocytic Lymphohistiocytosis in an Infant: Important Aspects in Management

WH Hui, DKK Ng, KL Kwok, YY Lam


Haemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation that carries a poor prognosis. We reported a case of HLH in an infant that had a complete remission after receiving intravenous immunoglobulin (IVIG) and highlighted the important aspects in the management of such disease. Diagnosis of HLH should always be considered early in patients with unremitting fever, hepatosplenomegaly and cytopenias. Neurological involvement is common and lumbar puncture is recommended in all cases if clinical condition allows. NK cell function should be checked in all cases and helps to differentiate primary and secondary HLH. IVIG, corticosteroid, cyclosporine A and etoposide have all been suggested as treatment options of HLH. The HLH protocol should be started without delay for severe disease or primary HLH. For mild non-familial disease, IVIG had been suggested by some authors as the initial treatment although confirmed evidence is still lacking. Further studies are necessary to compare the efficacy of different treatment options. It is hoped that early diagnosis and intervention will increase the chance of survival.

Keyword : Haemophagocytic lymphohistiocytosis; Infant; Intravenous immunoglobulin; Management

Abstract in Chinese


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