Extract

Department of Paediatric Rheumatology, 1Department of Paediatric Haematology and 2Department of Paediatric Histopathology, Royal Manchester Children's Hospital, Manchester, UK

Sir, We thank Dr Jawad for his comments and his interest in our paper [1]. To address the issues raised by the author, there was no family history of haemophagocytic lymphohistiocytosis (HLH) or of consanguinity. We did do serology for Epstein–Barr virus (EBV) and parvovirus, both of which were negative (we mentioned in our paper that the viral serology was negative, although we did not specify the viruses checked).

As for his comments on the diagnostic criteria, these are beset with problems. The criteria were designed primarily for the diagnosis of primary HLH, but many clinicians in practice use the criteria for secondary HLH and for HLH associated with rheumatic disease. Henter et al. [2], in their criteria, acknowledge the fact that not all patients will fulfil the criteria and that clinical decisions regarding therapy need to be made even when patients do not satisfy the diagnostic criteria.

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