Curr Oncol Rep. 2003 Nov;5(6):510-5.

Juvenile myelomonocytic leukemia.

Source

Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Mathildenstrasse 1, 79106 Freiburg, Germany. Niemeyer@kikli.ukl.uni-freiburg.de

Abstract

Juvenile myelomonocytic leukemia is an aggressive neoplasia of early childhood. Only allogeneic stem cell transplantation (SCT) offers long-term cure. In the absence of an HLA-matched family donor, early SCT from an unrelated donor is the treatment of choice for most children. With clear evidence of a graft-versus-leukemia effect and a high post-transplant relapse rate, the outcome of SCT depends, in part, on the management of immunosuppression during the procedure. The impact of pretransplant cytoreductive treatment, such as intensive chemotherapy, splenectomy, or 13-cis retinoic acid, is unclear. Hypersensitivity for granulocyte-macrophage colony-stimulating factor and pathologic activation of the Ras/MAPK pathway play an important role in the pathophysiology of juvenile myelomonocytic leukemia and provide the opportunity for several novel therapeutic approaches.

PMID:: 14521811; [PubMed - indexed for MEDLINE]

Publication Types, MeSH Terms, Substances

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Review

MeSH Terms

Substances

Granulocyte-Macrophage Colony-Stimulating Factor

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Labome Researcher Resource - ExactAntigen/Labome

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Juvenile Myelomonocytic Leukemia - Genetic Alliance

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Related citations in PubMed

Review Juvenile myelomonocytic leukemia. [Curr Treat Options Oncol. 2003]
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Cited by 1 PubMed Central article

The mutational spectrum of PTPN11 in juvenile myelomonocytic leukemia and Noonan syndrome/myeloproliferative disease. [Blood. 2005]
The mutational spectrum of PTPN11 in juvenile myelomonocytic leukemia and Noonan syndrome/myeloproliferative disease.
Kratz CP, Niemeyer CM, Castleberry RP, Cetin M, Bergsträsser E, Emanuel PD, Hasle H, Kardos G, Klein C, Kojima S, et al. Blood. 2005 Sep 15; 106(6):2183-5. Epub 2005 May 31.

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