Acute lymphoblastic leukemia with t(4;11) in children 1 year and older: The 'big sister' of the infant disease?
Leukemia
; 21(4): 642-6, 2007 Apr.
Article
in En
| MEDLINE
| ID: mdl-17287854
ABSTRACT
The t(4;11)-positive acute lymphoblastic leukemia (ALL) is a rare disease in children above the age of 1 year. We studied the clinical and biological characteristics in 32 consecutively diagnosed childhood cases (median age 10.0 years, range 1.0-17.1 years). Immunophenotyping revealed a pro-B and a pre-B stage in 24 and eight cases, respectively. IGH genes were rearranged in 84% of leukemias with a predominance of incomplete DJ(H) joints. Whereas IGK-Kde and TCRD rearrangements were rare, TCRG rearrangements were present in 50% of cases and involved mainly Vgamma11 or Vgamma9 together with a Jgamma1.3./2.3 gene segment, an unusual combination among t(4;11)-negative B-cell precursor ALL. Oligoclonality was found in about 30% as assessed by heterogeneous IGH and TCRG rearrangements. Our data are in line with transformation of a precursor cell at an early stage of B-cell development but retaining the potential to differentiate to the pre-B cell stage in vivo. Although a distinct difference between infant and older childhood cases with t(4;11) became evident, no age-related biological features were found within the childhood age group. In contrast to infants with t(4;11)-positive ALL, childhood cases had a relatively low cumulative incidence of relapse of 25% at 3.5 years with BFM-based high-risk protocols.
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Database:
MEDLINE
Main subject:
Translocation, Genetic
/
Chromosomes, Human, Pair 4
/
Chromosomes, Human, Pair 11
/
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Type of study:
Guideline
Limits:
Humans
/
Infant
Language:
En
Journal:
Leukemia
Journal subject:
HEMATOLOGIA
/
NEOPLASIAS
Year:
2007
Type:
Article
Affiliation country:
Austria