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Haemophagocytic lymphohistiocytosis in children
Author(s) -
Hallahan AR,
Carpenter PA,
O’GormanHughes DW,
Vowels MR,
Marshall GM
Publication year - 1999
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1046/j.1440-1754.1999.t01-1-00354.x
Subject(s) - medicine , pancytopenia , hepatosplenomegaly , hemophagocytic lymphohistiocytosis , pediatrics , disease , context (archaeology) , transplantation , bone marrow , surgery , paleontology , biology
Objective: To evaluate the clinical and diagnostic features of children presenting with haemophagocytic lymphohistiocytosis (HLH), evolution of the disease and outcomes in response to treatment. Methodology: The medical records of 12 children, aged 5 weeks to 13 years at diagnosis, with HLH managed at a single institution were reviewed. Results: Presenting features were fever, hepatosplenomegaly, pancytopenia and hypertriglyceridemia or hypofibrinogenemia. Nine patients (75%) developed central nervous system (CNS) disease. Only one child with CNS disease survived. Five children had complete responses to therapy (42%), but all relapsed at a median of 1.5 months after starting treatment (range 2 weeks to 5 months). Two of the children treated are long‐term survivors (17%), both after allogeneic bone marrow transplantation. All deaths occurred in the context of active disease. Conclusions: Haemophagocytic lymphohistiocytosis is a disease with a poor prognosis. Central nervous system complications are common and response to treatment usually is transient. This study provides support for the use of immuno‐ modulatory therapy for remission introduction followed by consideration of allogeneic bone marrow transplantation.