Document Detail

Chronic myeloid leukemia in an adolescent with Ollier's disease after intensive X-ray exposure.
MedLine Citation:
PMID:  15160927     Owner:  NLM     Status:  MEDLINE    
We report a 19-year-old man with Ollier's disease with multiple orthopedic procedures performed for leg length discrepancy; who developed chronic myeloid leukemia presenting with intramuscular hematoma. His symptoms resolved with cytoreductive treatment by hydroxyurea. Cytogenetic and molecular investigations showed a complex Philadelphia translocation t(9;22;13) (q34;q11.2;q12), with predominance of ela2 BCR/ABL splicing and deletion of reciprocal der(9) ABL/BCR locus, all suggesting poor prognosis. The cumulative X-ray exposure from repeated operations from the age of 7 to 12 years was estimated to be around 16 mSv, approximately the dose of 720 chest X rays. Literature review showed two other cases of leukemia occurring in patients with multiple enchondromatosis. Although the development of CML in this young patient might be related partly to genetic defects, the repeated radiation exposure, especially at young age and directly on the marrow tissue in the long bones, might also be an important pathogenetic factor.
W Y Au; G C Ooi; S K Ma; T S K Wan; Y L Kwong
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Leukemia & lymphoma     Volume:  45     ISSN:  1042-8194     ISO Abbreviation:  Leuk. Lymphoma     Publication Date:  2004 Mar 
Date Detail:
Created Date:  2004-05-26     Completed Date:  2004-07-09     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9007422     Medline TA:  Leuk Lymphoma     Country:  England    
Other Details:
Languages:  eng     Pagination:  613-6     Citation Subset:  IM    
Department of Medicine, Queen Mary Hospital, University of Hong Kong, SAR, China.
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MeSH Terms
Chromosome Aberrations
Cytogenetic Analysis
Enchondromatosis / complications*,  radiography*,  surgery
Leukemia, Myelogenous, Chronic, BCR-ABL Positive / etiology*
Radiation Dosage
X-Rays / adverse effects*

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