Document Detail


FLT3-internal tandem duplication in a pediatric patient with t(8;21) acute myeloid leukemia.
MedLine Citation:
PMID:  21156247     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Patients diagnosed with t(8;21)-acute myeloid leukemia (AML) are currently considered to have good prognoses, but about half of these patients relapse. FLT3-internal tandem duplication (ITD) is generally thought to be strongly associated with poor prognosis in AML, but is rarely reported in patients with t(8;21)-AML. Expression of the neural cell-adhesion molecule (CD56) is also associated with a significantly shorter complete remission duration and survival in patients with t(8;21)-AML. Patients with t(8;21)-AML expressing CD56 have been reported to exhibit a higher incidence of granulocytic sarcoma (GS), and t(8;21)-AML with GS results in a less favorable prognosis than AML with this translocation alone. Here, we report on a 15-year-old girl with t(8;21)-AML having both CD56 expression and FLT3-ITD. This patient underwent unrelated donor bone marrow transplantation and achieved complete remission, but thereafter presented with obstructive jaundice caused by GS compression of the common bile duct without bone marrow invasion at relapse. Autopsy revealed multiple nodules of the stomach membrane and invasion into the head of the pancreas. For earlier detection of relapse, we suggest that it would be useful to examine existence of GS in CD56-positive t(8;21)-AML patients at diagnosis and hematologic remission. Even though t(8;21)-AML is less likely to co-occur with FLT3-ITD in pediatric patients, this report suggests that prognostic factors, including FLT3 and KIT genes and the surface marker CD56, should be analyzed in these patients.
Authors:
Machiko Kawamura; Hidefumi Kaku; Tateki Ito; Nobuaki Funata; Tomohiko Taki; Akira Shimada; Yasuhide Hayashi
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Cancer genetics and cytogenetics     Volume:  203     ISSN:  1873-4456     ISO Abbreviation:  Cancer Genet. Cytogenet.     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-15     Completed Date:  2011-01-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7909240     Medline TA:  Cancer Genet Cytogenet     Country:  United States    
Other Details:
Languages:  eng     Pagination:  292-6     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier Inc. All rights reserved.
Affiliation:
Department of Pediatrics, Tokyo Metropolitan Cancer and Infectious Disease Center Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. m.kawamura@cick.jp
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Antigens, CD56 / biosynthesis
Chromosomes, Human, Pair 21*
Chromosomes, Human, Pair 8*
Fatal Outcome
Female
Gene Duplication*
Humans
Jaundice, Obstructive / mortality,  pathology
Leukemia, Myeloid, Acute / complications,  genetics*,  mortality
Male
Middle Aged
Proto-Oncogene Proteins c-kit / metabolism
Recurrence
Remission Induction
Translocation, Genetic*
Chemical
Reg. No./Substance:
0/Antigens, CD56; EC 2.7.10.1/Proto-Oncogene Proteins c-kit

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


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