MYC translocation in chronic lymphocytic leukaemia is associated with increased prolymphocytes and a poor prognosis.
Chromosomal translocations that involve MYC, characteristic of Burkitt lymphoma, are rare in chronic lymphocytic leukaemia (CLL). We report the clinical, morphological, immunophenotypic, cytogenetic and molecular genetic features of eight CLL cases with MYC rearrangement. The patients, five men and three women (median age, 71 years) had bone marrow involvement and an absolute peripheral blood lymphocytosis; five had lymphadenopathy; seven had splenomegaly. Prolymphocytes were increased (>/=10%) in all cases. Six cases were classified as CLL with increased prolymphocytes (CLL/PL; prolymphocytes 10-55%), and two were classified as CLL in prolymphocytic transformation (CLL/PT; prolymphocytes >55%). All cases co-expressed CD5, CD19, and CD23; five of eight expressed ZAP-70. Of seven cases tested, four had mutated and three had unmutated IGHV genes. Conventional cytogenetic studies demonstrated t(8;14)(q24.1;q32) in five cases, t(8;22)(q24.1;q11) in two cases, and t(2;8)(p12;q24.1) in one case. Seven cases contained additional chromosomal abnormalities. All patients received combination chemotherapy. Two developed Epstein-Barr virus (EBV)-associated diffuse large B-cell lymphomas (DLBCL) that were clonally unrelated to the CLL. At follow-up, two patients are alive, four died of underlying disease, one died of EBV-associated DLBCL, and one died of an unrelated cancer. In summary, MYC rearrangement, which occurs rarely in CLL patients, is associated with increased prolymphocytes, complex cytogenetic abnormalities, and a poor prognosis.
Huh
Yang O
YO
Department of Hematopathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA. yhuh@mdanderson.org
Lin
Katherine I-Chun
KI
Vega
Francisco
F
Schlette
Ellen
E
Yin
C Cameron
CC
Keating
Michael J
MJ
Luthra
R
R
Medeiros
L Jeffrey
LJ
Abruzzo
Lynne V
LV
D000368:Aged; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology; D005260:Female; D016259:Genes, myc / Q000235:genetics*; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics* / Q000473:pathology; D054503:Lymphoid Progenitor Cells / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D011379:Prognosis; D014178:Translocation, Genetic / Q000235:genetics*