Segmental tandem triplication of the MLL gene in an intravascular large B-cell lymphoma with multisystem involvement

A comprehensive morphologic, immunophenotypic, cytogenetic, and molecular cytogenetic antemortem study

Jeremy Deisch, Franklin Fuda, Weina Chen, Nitin Karandikar, Arnaldo A. Arbini, Xin J. Zhou, Huan You Wang

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Abstract

An association between intravascular large B-cell lymphoma (IVLBCL) and the mixed lineage leukemia (MLL) gene has never been demonstrated. Here, we report an IVLBCL in a 47-year-old Asian man. Morphologically, the atypical lymphoid infiltrate was entirely confined in the lumina of capillaries, small vessels, and sinusoidal space. Within the kidney, the neoplastic lymphoid cells exhibited both the glomerular and peritubular capillary distribution pattern. Conventional cytogenetic analysis from the bone marrow aspirates displayed a complex karyotype, with a notable triple tandem repeat at band segment q22-q25 of chromosome 11. Fluorescence in situ hybridization with an MLL probe set, performed on both interphase cells and metaphase spreads, confirmed the presence of 3 copies of the MLL gene on the derivative chromosome 11. From this finding and 3 other IVLBCL cases reported in the literature, we conclude that MLL may play an important role in the lymphomagenesis of IVLBCL at least in a subset of cases.

Original languageEnglish (US)
Pages (from-to)1477-1482
Number of pages6
JournalArchives of Pathology and Laboratory Medicine
Volume133
Issue number9
StatePublished - Sep 2009

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B-Cell Lymphoma
Cytogenetics
Leukemia
Chromosomes, Human, Pair 11
Genes
Tandem Repeat Sequences
Cytogenetic Analysis
Interphase
Metaphase
Fluorescence In Situ Hybridization
Karyotype
Bone Marrow
Lymphocytes
Kidney

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

Cite this

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title = "Segmental tandem triplication of the MLL gene in an intravascular large B-cell lymphoma with multisystem involvement: A comprehensive morphologic, immunophenotypic, cytogenetic, and molecular cytogenetic antemortem study",
abstract = "An association between intravascular large B-cell lymphoma (IVLBCL) and the mixed lineage leukemia (MLL) gene has never been demonstrated. Here, we report an IVLBCL in a 47-year-old Asian man. Morphologically, the atypical lymphoid infiltrate was entirely confined in the lumina of capillaries, small vessels, and sinusoidal space. Within the kidney, the neoplastic lymphoid cells exhibited both the glomerular and peritubular capillary distribution pattern. Conventional cytogenetic analysis from the bone marrow aspirates displayed a complex karyotype, with a notable triple tandem repeat at band segment q22-q25 of chromosome 11. Fluorescence in situ hybridization with an MLL probe set, performed on both interphase cells and metaphase spreads, confirmed the presence of 3 copies of the MLL gene on the derivative chromosome 11. From this finding and 3 other IVLBCL cases reported in the literature, we conclude that MLL may play an important role in the lymphomagenesis of IVLBCL at least in a subset of cases.",
author = "Jeremy Deisch and Franklin Fuda and Weina Chen and Nitin Karandikar and Arbini, {Arnaldo A.} and Zhou, {Xin J.} and Wang, {Huan You}",
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AU - Deisch, Jeremy

AU - Fuda, Franklin

AU - Chen, Weina

AU - Karandikar, Nitin

AU - Arbini, Arnaldo A.

AU - Zhou, Xin J.

AU - Wang, Huan You

PY - 2009/9

Y1 - 2009/9

N2 - An association between intravascular large B-cell lymphoma (IVLBCL) and the mixed lineage leukemia (MLL) gene has never been demonstrated. Here, we report an IVLBCL in a 47-year-old Asian man. Morphologically, the atypical lymphoid infiltrate was entirely confined in the lumina of capillaries, small vessels, and sinusoidal space. Within the kidney, the neoplastic lymphoid cells exhibited both the glomerular and peritubular capillary distribution pattern. Conventional cytogenetic analysis from the bone marrow aspirates displayed a complex karyotype, with a notable triple tandem repeat at band segment q22-q25 of chromosome 11. Fluorescence in situ hybridization with an MLL probe set, performed on both interphase cells and metaphase spreads, confirmed the presence of 3 copies of the MLL gene on the derivative chromosome 11. From this finding and 3 other IVLBCL cases reported in the literature, we conclude that MLL may play an important role in the lymphomagenesis of IVLBCL at least in a subset of cases.

AB - An association between intravascular large B-cell lymphoma (IVLBCL) and the mixed lineage leukemia (MLL) gene has never been demonstrated. Here, we report an IVLBCL in a 47-year-old Asian man. Morphologically, the atypical lymphoid infiltrate was entirely confined in the lumina of capillaries, small vessels, and sinusoidal space. Within the kidney, the neoplastic lymphoid cells exhibited both the glomerular and peritubular capillary distribution pattern. Conventional cytogenetic analysis from the bone marrow aspirates displayed a complex karyotype, with a notable triple tandem repeat at band segment q22-q25 of chromosome 11. Fluorescence in situ hybridization with an MLL probe set, performed on both interphase cells and metaphase spreads, confirmed the presence of 3 copies of the MLL gene on the derivative chromosome 11. From this finding and 3 other IVLBCL cases reported in the literature, we conclude that MLL may play an important role in the lymphomagenesis of IVLBCL at least in a subset of cases.

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