MYC translocation in chronic lymphocytic leukaemia is associated with increased prolymphocytes and a poor prognosis

Yang O. Huh, Katherine I Chun Lin, Francisco Vega, Ellen Schlette, C. Cameron Yin, Michael J. Keating, R. Luthra, L. Jeffrey Medeiros, Lynne V. Abruzzo

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish
Pages (from-to)36-44
Number of pages9
JournalBritish Journal of Haematology
Volume142
Issue number1
DOIs
StatePublished - Jul 1 2008
Externally publishedYes

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B-Cell Chronic Lymphocytic Leukemia
Lymphoma, Large B-Cell, Diffuse
Human Herpesvirus 4
Cytogenetics
Chromosome Aberrations
Lymphocytosis
Genetic Translocation
Burkitt Lymphoma
Splenomegaly
Combination Drug Therapy
Molecular Biology
Bone Marrow
Genes

Keywords

  • Chronic lymphocytic leukaemia
  • Epstein-Barr virus
  • MYC rearrangement
  • Prolymphocytes
  • Transformation

ASJC Scopus subject areas

  • Hematology

Cite this

MYC translocation in chronic lymphocytic leukaemia is associated with increased prolymphocytes and a poor prognosis. / Huh, Yang O.; Lin, Katherine I Chun; Vega, Francisco; Schlette, Ellen; Yin, C. Cameron; Keating, Michael J.; Luthra, R.; Medeiros, L. Jeffrey; Abruzzo, Lynne V.

In: British Journal of Haematology, Vol. 142, No. 1, 01.07.2008, p. 36-44.

Research output: Contribution to journalArticle

Huh, Yang O. ; Lin, Katherine I Chun ; Vega, Francisco ; Schlette, Ellen ; Yin, C. Cameron ; Keating, Michael J. ; Luthra, R. ; Medeiros, L. Jeffrey ; Abruzzo, Lynne V. / MYC translocation in chronic lymphocytic leukaemia is associated with increased prolymphocytes and a poor prognosis. In: British Journal of Haematology. 2008 ; Vol. 142, No. 1. pp. 36-44.
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T1 - MYC translocation in chronic lymphocytic leukaemia is associated with increased prolymphocytes and a poor prognosis

AU - Huh, Yang O.

AU - Lin, Katherine I Chun

AU - Vega, Francisco

AU - Schlette, Ellen

AU - Yin, C. Cameron

AU - Keating, Michael J.

AU - Luthra, R.

AU - Medeiros, L. Jeffrey

AU - Abruzzo, Lynne V.

PY - 2008/7/1

Y1 - 2008/7/1

N2 - 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.

AB - 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.

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KW - Epstein-Barr virus

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KW - Prolymphocytes

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