Document Detail


Use of human androgen receptor gene analysis to aid the diagnosis of JMML in female noonan syndrome patients.
MedLine Citation:
PMID:  18454468     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Noonan syndrome (NS) patients are at increased risk for developing juvenile myelomonocytic leukemia (JMML), an aggressive clonal disorder of aberrant cell proliferation. Many NS patients exhibit spontaneously remitting monocytosis and transient myeloproliferation. The distinction between bone marrow hyperproliferation due to germline mutation and leukemia resulting from clonal transformation can be difficult in NS patients. The GM-CSF hypersensitivity assay, diagnostic of sporadic JMML, can be positive in NS patients at baseline. In this report, we demonstrate the utility of determining the clonal status of the monocyte population by the HUMARA assay in distinguishing JMML and benign myeloproliferation in female NS patients.
Authors:
Viviana A Lavin; Rizwan Hamid; Johnequia Patterson; Catherine Alford; Richard Ho; Elizabeth Yang
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric blood & cancer     Volume:  51     ISSN:  1545-5017     ISO Abbreviation:  Pediatr Blood Cancer     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-06-12     Completed Date:  2008-06-24     Revised Date:  2009-01-12    
Medline Journal Info:
Nlm Unique ID:  101186624     Medline TA:  Pediatr Blood Cancer     Country:  United States    
Other Details:
Languages:  eng     Pagination:  298-302     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Divisions of Hematology-Oncology, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA.
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MeSH Terms
Descriptor/Qualifier:
Female
Granulocyte-Macrophage Colony-Stimulating Factor / pharmacology
Humans
Infant
Leukemia, Myelomonocytic, Juvenile / diagnosis*
Mutation
Noonan Syndrome / complications*
Protein Tyrosine Phosphatase, Non-Receptor Type 11 / genetics
Receptors, Androgen / genetics*
Chemical
Reg. No./Substance:
0/AR protein, human; 0/Receptors, Androgen; 83869-56-1/Granulocyte-Macrophage Colony-Stimulating Factor; EC 3.1.3.48/PTPN11 protein, human; EC 3.1.3.48/Protein Tyrosine Phosphatase, Non-Receptor Type 11

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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