期刊
PEDIATRIC BLOOD & CANCER
卷 52, 期 2, 页码 268-272出版社
WILEY-LISS
DOI: 10.1002/pbc.21790
关键词
Chediak-Higashi syndrome; etoposide; Griscelli syndrome; HLH; X-linked lymphoproliferative disease
资金
- Children's Cancer Foundation of Sweden
- Cancer and Allergy Foundation of Sweden
- Swedish Research Council
- Marta and Gunnar V Philipson Foundation
- Stockholm County Council
Background. Griscelli syndrome type 2 (GS2), the X-linked lymphoproliferative (XLP) and the Chediak-Higashi (CHS) syndromes are diseases that all may develop hemophagocytic syndromes. We wanted to investigate whether the treatment protocols for hemophagocytic lymphohistiocytosis (HLH) can also be used for these syndromes. Procedure. In the HLH-94/HLH-2004 treatment Study registries, we evaluated all patients with GS2 (n = 5), XLP (n = 2) or CHS (n = 2) treated between 1994 and 2004. Results. All patients responded to the therapy, and all are alive but one (suffering from CHS), with a mean follow-up) of 5.6 years. All GS2, one XLP and one CHS patient underwent hematopoietic stein cell transplant. Mean follow-up post transplant was 6.0 years. Six of the seven transplanted children achieved non-active disease Status at the time for SCT. Neurological sequelae were reported in all, except for the XLP patients. Conclusions. Our results indicate that HLH treatment can be an effective first line treatment to induce remission in patients with GS2, XLP and CHS that have developed a hemophagocytic syndrome. We suggest that these patients should be included as a separate cohort in the international HLH study. Pediatr Blood Cancer 2009;52:268-272. (C) 2008 Wiley-Liss, Inc.
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