Intermediate lymphocytic lymphoma

Clinical and pathologic features of a recently characterized subtype of non-hodgkin's lymphoma

Ramesh A. Shivdasani, Jay Hess, Arthur T. Skarin, Geraldine S. Pinkus

Research output: Contribution to journalArticle

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Abstract

Purpose: We present a comprehensive review of clinical, pathologic, molecular, and prognostic features and therapy of intermediate lymphocytic (mantle cell) lymphoma (ILL/MCL), a recently characterized subtype that represents 2% to 8% of non-Hodgkin's lymphomas (NHLs), but which has not been included in most classification schemes, including the International Working Formulation. Design: The English-language literature encompassing the above aspects, published between 1977 and 1992, is critically reviewed. Results and Conclusion: ILL/MCL is a disease of proliferating B lymphocytes that is characterized by generalized lymphadenopathy and frequent, often extensive, involvement of the spleen, bone marrow, and gastrointestinal tract. The malignant cells usually express the markers CD5 and IgM with or without IgD, but not CD10, on the cell surface, and grow in one of two dominant histologic patterns: mantle zone and diffuse. The characteristic cytogenetic abnormality is a t(11;14)(q13;q32) translocation, which juxtaposes the bcl-1 locus on chromosome 11 with the immunoglobulin (Ig) heavy-chain locus on chromosome 14, and appears to result in dysregulated expression of the gene encoding cyclin D1. Median survival is in the range of 2 to 5 years. While responses to chemotherapy may be seen in up to half the patients, relapses are the rule, and longterm survival is uncommon. The optimal treatment remains undefined, although therapy may be deferred until there are symptoms or complications, at which time judicious administration of alkylating agents and glucocorticoids may result in effective palliation.

Original languageEnglish (US)
Pages (from-to)802-811
Number of pages10
JournalJournal of Clinical Oncology
Volume11
Issue number4
StatePublished - 1993
Externally publishedYes

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Mantle-Cell Lymphoma
Non-Hodgkin's Lymphoma
bcl-1 Genes
Chromosomes, Human, Pair 14
Immunoglobulin D
Immunoglobulin Heavy Chains
Chromosomes, Human, Pair 11
Survival
Alkylating Agents
Chromosome Aberrations
Glucocorticoids
Immunoglobulin M
Gastrointestinal Tract
B-Lymphocytes
Language
Therapeutics
Spleen
Bone Marrow
Recurrence
Drug Therapy

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Intermediate lymphocytic lymphoma : Clinical and pathologic features of a recently characterized subtype of non-hodgkin's lymphoma. / Shivdasani, Ramesh A.; Hess, Jay; Skarin, Arthur T.; Pinkus, Geraldine S.

In: Journal of Clinical Oncology, Vol. 11, No. 4, 1993, p. 802-811.

Research output: Contribution to journalArticle

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