Follicular centre cell lymphoma with alpha heavy chain disease - A histopathological and immunohistological study

Z. Nemes, V. Thomázy, G. Szeifert

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15 Citations (Scopus)

Abstract

The first recorded case of a small intestinal lymphoma with alpha heavy chain disease occurring in Hungary is reported. The clinical manifestation of the disease and the focal distribution of mucosal alterations do not fulfill the criteria to make a diagnosis of Mediterranean type lymphoma (MTL) but the lymphomatous segments of the jejunum show the same pathological and immunohistological characteristics as seen in MTL. One of the basic features of MTL, the so called lympho-histiocytic nodules, which have been suspected by previous authors to represent an incipient neoplastic process involving histiocytic cells, is identified as follicular centroblastic/centrocytic malignant lymphoma. The cytogenetical connection between the massive proliferation of abnormal alpha chain producing plasma cells and neoplastic germinal centres is substantiated by direct immunohistological evidence using a combined immunofluorescent and immunoperoxidase technique to detect heavy and light chains within the same cell. The sarcomatousappearing pleomorphic cell proliferation is interpreted as an anaplastic change in the centroblastic/centrocytic lymphoma. Unequivocal evidence for an abnormal IgA production in this pleomorphic component has not been obtained. Our observations suggest that in alpha heavy chain disease the neoplastic cell population originates in the germinal centres.

Original languageEnglish
Pages (from-to)119-132
Number of pages14
JournalVirchows Archiv A Pathological Anatomy and Histology
Volume394
Issue number1-2
DOIs
Publication statusPublished - Dec 1981

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Immunoproliferative Small Intestinal Disease
Heavy Chain Disease
Lymphoma
Germinal Center
Neoplastic Processes
Hungary
Jejunum
Plasma Cells
Immunoenzyme Techniques
Immunoglobulin A
Cell Proliferation
Light
Population

Keywords

  • Alpha chain disease
  • Histopathology
  • Immunohistology
  • Mediterranean type lymphoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Anatomy

Cite this

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abstract = "The first recorded case of a small intestinal lymphoma with alpha heavy chain disease occurring in Hungary is reported. The clinical manifestation of the disease and the focal distribution of mucosal alterations do not fulfill the criteria to make a diagnosis of Mediterranean type lymphoma (MTL) but the lymphomatous segments of the jejunum show the same pathological and immunohistological characteristics as seen in MTL. One of the basic features of MTL, the so called lympho-histiocytic nodules, which have been suspected by previous authors to represent an incipient neoplastic process involving histiocytic cells, is identified as follicular centroblastic/centrocytic malignant lymphoma. The cytogenetical connection between the massive proliferation of abnormal alpha chain producing plasma cells and neoplastic germinal centres is substantiated by direct immunohistological evidence using a combined immunofluorescent and immunoperoxidase technique to detect heavy and light chains within the same cell. The sarcomatousappearing pleomorphic cell proliferation is interpreted as an anaplastic change in the centroblastic/centrocytic lymphoma. Unequivocal evidence for an abnormal IgA production in this pleomorphic component has not been obtained. Our observations suggest that in alpha heavy chain disease the neoplastic cell population originates in the germinal centres.",
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N2 - The first recorded case of a small intestinal lymphoma with alpha heavy chain disease occurring in Hungary is reported. The clinical manifestation of the disease and the focal distribution of mucosal alterations do not fulfill the criteria to make a diagnosis of Mediterranean type lymphoma (MTL) but the lymphomatous segments of the jejunum show the same pathological and immunohistological characteristics as seen in MTL. One of the basic features of MTL, the so called lympho-histiocytic nodules, which have been suspected by previous authors to represent an incipient neoplastic process involving histiocytic cells, is identified as follicular centroblastic/centrocytic malignant lymphoma. The cytogenetical connection between the massive proliferation of abnormal alpha chain producing plasma cells and neoplastic germinal centres is substantiated by direct immunohistological evidence using a combined immunofluorescent and immunoperoxidase technique to detect heavy and light chains within the same cell. The sarcomatousappearing pleomorphic cell proliferation is interpreted as an anaplastic change in the centroblastic/centrocytic lymphoma. Unequivocal evidence for an abnormal IgA production in this pleomorphic component has not been obtained. Our observations suggest that in alpha heavy chain disease the neoplastic cell population originates in the germinal centres.

AB - The first recorded case of a small intestinal lymphoma with alpha heavy chain disease occurring in Hungary is reported. The clinical manifestation of the disease and the focal distribution of mucosal alterations do not fulfill the criteria to make a diagnosis of Mediterranean type lymphoma (MTL) but the lymphomatous segments of the jejunum show the same pathological and immunohistological characteristics as seen in MTL. One of the basic features of MTL, the so called lympho-histiocytic nodules, which have been suspected by previous authors to represent an incipient neoplastic process involving histiocytic cells, is identified as follicular centroblastic/centrocytic malignant lymphoma. The cytogenetical connection between the massive proliferation of abnormal alpha chain producing plasma cells and neoplastic germinal centres is substantiated by direct immunohistological evidence using a combined immunofluorescent and immunoperoxidase technique to detect heavy and light chains within the same cell. The sarcomatousappearing pleomorphic cell proliferation is interpreted as an anaplastic change in the centroblastic/centrocytic lymphoma. Unequivocal evidence for an abnormal IgA production in this pleomorphic component has not been obtained. Our observations suggest that in alpha heavy chain disease the neoplastic cell population originates in the germinal centres.

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