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Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis.

Abstract：

:Haemophagocytic lymphohistiocytosis (HLH) may cause meningoencephalitis and significant neurological sequelae. We examined the relationship between neurological symptoms and cerebrospinal fluid (CSF) at diagnosis, and long-term outcome, in all children enroled in the HLH-94-study prior to July 1, 2003, for whom information on CSF at diagnosis was available (n = 193). Patients were divided into four groups: (i) normal CSF (cells/protein) and no neurological symptoms (n = 71); (ii) normal CSF but neurological symptoms (n = 21); (iii) abnormal CSF but no symptoms (n = 50); and (iv) abnormal CSF with neurological symptoms (n = 51). At diagnosis, neurological symptoms were reported in 72/193 (37%) (seizures = 23); abnormal CSF in 101/193 (52%), and either or both in 122/193 (63%). Altogether 16/107 (15%) survivors had neurological sequelae at follow-up (median 5.3 years). Multivariate hazard ratios (HR) for mortality were 0.98 [95% confidence interval (CI) = 0.42-2.31], 1.52 (0.82-2.82) and 2.05 (1.13-3.72) for groups 2-4, compared with group 1. Moreover, sequelae were more frequent in group 4 (7/21, 33%) compared to groups 1-3 (9/86, 10%) (P = 0.015). Patients with abnormal CSF at diagnosis had significantly increased mortality [HR = 1.78 (95% CI = 1.08-2.92), P = 0.023]. Thus, a substantial proportion of HLH survivors suffer neurological sequelae, and children with abnormal CSF have increased risk of mortality and neurological sequelae. Prompt treatment of HLH at onset or relapse may reduce these complications.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

Horne A,Trottestam H,Aricò M,Egeler RM,Filipovich AH,Gadner H,Imashuku S,Ladisch S,Webb D,Janka G,Henter JI,Histiocyte Society.

doi

10.1111/j.1365-2141.2007.06922.x

subject

Has Abstract

pub_date

2008-02-01 00:00:00

pages

327-35

issue

3

eissn

0007-1048

issn

1365-2141

pii

BJH6922

journal_volume

140

pub_type

杂志文章,多中心研究

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