| Treatment of the X-linked lymphoproliferative, Griscelli and Chédiak-Higashi syndromes by HLH directed therapy. | |
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MedLine Citation:
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PMID: 18937330 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Griscelli syndrome type 2 (GS2), the X-linked lymphoproliferative (XLP) and the Chédiak-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 stem 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. |
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Authors:
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Helena Trottestam; Karin Beutel; Marie Meeths; Niels Carlsen; Carsten Heilmann; Srdjan Pasić; David Webb; Henrik Hasle; Jan-Inge Henter |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric blood & cancer Volume: 52 ISSN: 1545-5017 ISO Abbreviation: Pediatr Blood Cancer Publication Date: 2009 Feb |
Date Detail:
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Created Date: 2008-12-16 Completed Date: 2009-02-06 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101186624 Medline TA: Pediatr Blood Cancer Country: United States |
Other Details:
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Languages: eng Pagination: 268-72 Citation Subset: IM |
Copyright Information:
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(c) 2008 Wiley-Liss, Inc. |
Affiliation:
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Childhood Cancer Research Unit, Karolinska University Hospital, Department of Woman and Child Health, Karolinska Institutet, Stockholm, Sweden. Helena.Trottestam@ki.se |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Chediak-Higashi Syndrome Child Female Hematopoietic Stem Cell Transplantation Humans Lymphohistiocytosis, Hemophagocytic / therapy* Lymphoproliferative Disorders / therapy* Male Registries Remission Induction Retrospective Studies Survival Rate Treatment Outcome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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