| Biphenotypic hematologic malignancy: a case report of the 8p11 myeloproliferative syndrome in a child. | |
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MedLine Citation:
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PMID: 20562652 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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SUMMARY: The 8p11 myeloproliferative syndrome, also known as stem cell leukemia/lymphoma, is a rare, atypical, myeloproliferative disorder and lymphoid malignancy associated with chromosomal abnormalities involving the 8p11 chromosomal band. Translocations associated with this syndrome result in the fusion of the fibroblast growth factor receptor 1 (FGFR 1) gene with various partners, resulting in ligand-independent FGFR activity. To date, 8 partner genes have been identified in association with FGFR1 rearrangements. The most frequent FGFR1 translocation partner is the zinc finger gene ZNF198 located at 13q11. Disease phenotypes associated with this translocation include poor prognosis and transformation to acute leukemia and non-Hodgkin lymphoma. In common with a T-cell phenotype, obtaining and maintaining remission is difficult by conventional chemotherapy. This study describes an illustrative case of 8p11 myeloproliferative syndrome/stem cell leukemia/lymphoma outlining its chief features and historical developments. |
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Authors:
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Xiangli Chen; Yin Zhang; Yulong Li; Pingchong Lei; Yaping Zhai; Linxiang Liu |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of pediatric hematology/oncology Volume: 32 ISSN: 1536-3678 ISO Abbreviation: J. Pediatr. Hematol. Oncol. Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-08-06 Completed Date: 2010-08-20 Revised Date: 2011-10-06 |
Medline Journal Info:
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Nlm Unique ID: 9505928 Medline TA: J Pediatr Hematol Oncol Country: United States |
Other Details:
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Languages: eng Pagination: 501-3 Citation Subset: IM |
Affiliation:
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Department of Clinical Hematology, Henan Provincial People's Hospital, First Hospital of Zhengzhou University, Zhengzhou, Henan Province, China. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Antineoplastic Combined Chemotherapy Protocols
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therapeutic use Child Chromosomes, Human, Pair 13 / genetics* Chromosomes, Human, Pair 8 / genetics* Cyclophosphamide / therapeutic use Dexamethasone / therapeutic use Doxorubicin / therapeutic use Etoposide / therapeutic use Humans Male Myeloproliferative Disorders / congenital, genetics*, physiopathology Phenotype Precancerous Conditions / congenital, genetics* Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / drug therapy, genetics, physiopathology Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / drug therapy, genetics, physiopathology Prednisone / therapeutic use Syndrome Translocation, Genetic Vincristine / therapeutic use |
| Chemical | |
Reg. No./Substance:
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23214-92-8/Doxorubicin; 33419-42-0/Etoposide; 50-02-2/Dexamethasone; 50-18-0/Cyclophosphamide; 53-03-2/Prednisone; 57-22-7/Vincristine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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