Chronic lymphocytic leukemia patients with highly stable and indolent disease show distinctive phenotypic and genotypic features.

Abstract:

:Different biologic features have been associated with a more or less aggressive clinical course in chronic lymphocytic leukemia (CLL). In the present study, 20 patients with highly stable CLL observed at a single institution over a period of 10 to 23 years and who never required treatment were extensively characterized. The aim was to identify a distinct and reproducible biologic profile associated with disease stability that may be used to recognize at presentation CLL patients who are likely to have a very benign clinical course and for whom treatment is not indicated. The results obtained indicate that numerous parameters are closely associated with disease stability: a typical CLL morphology and immunophenotype, the lack of expression of the CD38 antigen, the mutated immunoglobulin (Ig) heavy (H) chain variable (V) pattern, the absence of p53 mutations, a CD4/CD8 ratio more than 1, the lack of 17p and 11q deletions and of complex karyotypic aberrations, and the occurrence of the 13q14 deletion. No case displayed the VH3-21 gene, linked in mutated CLL with a poor outcome. In addition, the VH1-69 gene associated with unmutated CLL cases was never detected. These biologic features were coupled with an indolent clinical course characterized by an unmodified clinical stage over time, and by lack of autoimmune phenomena and of major infections requiring parental antibiotics. At a time when aggressive therapeutic strategies are always more frequently used in the management of CLL, the distinctive features of patients with long-lived stable disease should be prospectively identified at presentation.

journal_name

Blood

journal_title

Blood

authors

Guarini A,Gaidano G,Mauro FR,Capello D,Mancini F,De Propris MS,Mancini M,Orsini E,Gentile M,Breccia M,Cuneo A,Castoldi G,Foa R

doi

10.1182/blood-2002-12-3639

subject

Has Abstract

pub_date

2003-08-01 00:00:00

pages

1035-41

issue

3

eissn

0006-4971

issn

1528-0020

pii

2002-12-3639

journal_volume

102

pub_type

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