Document Detail

Prognostic implications of breakpoint and lineage heterogeneity in Philadelphia-positive acute lymphoblastic leukemia: a review.
MedLine Citation:
PMID:  8426467     Owner:  NLM     Status:  MEDLINE    
Philadelphia-positive (Ph) acute lymphoblastic leukemia (ALL) is heterogeneous both in terms of the BCR gene breakpoints (M-bcr and m-bcr) and in the number of cell lineages carrying the Ph chromosome. The impact of these observations on the controversy surrounding Ph ALL and Ph+ chronic granulocytic leukemia (CGL) is unclear. Twenty cases of Ph ALL (four newly investigated and 16 previously published) were classified into nine stem-cell (the Ph in myeloid and lymphoid cells) and 11 lymphoid-restricted cases. Lymphoid cases had a lower leucocyte count (56 x 10(9)/l) than stem-cell cases (151 x 10(9)/l) (p < 0.01). The M-bcr and m-bcr breakpoints (in 14 cases) were found in lymphoid (four cases of each) and stem-cell (five and one cases respectively) cases. Lymphoid cases had significantly shorter event-free survival (median 4 months) than stem-cell cases (median 35 months) (p < 0.01). M-bcr cases were older (mean 41 years) than m-bcr cases (mean 36 years)(p = NS); m-bcr cases had higher leukocyte counts (mean 85 x 10(9)/l) than M-bcr cases (36 x 10(9)/l)(p < 0.01) but breakpoint had no impact on prognosis. Lineage involvement, but not breakpoint, appears to distinguish prognostically important sub-groups of Ph ALL. Paradoxically, lymphoid-restricted cases have a worse prognosis than cases arising in a pluripotent stem-cell.
L M Secker-Walker; J M Craig
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Leukemia     Volume:  7     ISSN:  0887-6924     ISO Abbreviation:  Leukemia     Publication Date:  1993 Feb 
Date Detail:
Created Date:  1993-03-01     Completed Date:  1993-03-01     Revised Date:  2013-03-04    
Medline Journal Info:
Nlm Unique ID:  8704895     Medline TA:  Leukemia     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  147-51     Citation Subset:  IM    
Department of Haematology, Royal Free Hospital and School of Medicine, London, UK.
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MeSH Terms
Blast Crisis / genetics,  pathology
Child, Preschool
Chromosomes, Human, Pair 22*
Chromosomes, Human, Pair 9*
Middle Aged
Neoplastic Stem Cells / pathology*
Philadelphia Chromosome*
Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*,  mortality,  pathology
Survival Analysis
Translocation, Genetic / genetics*

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