New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies.

Journal Article (Journal Article)

Alpha Heavy Chain Disease has generally been described in the Mediterranean area in young people with a diffuse gastrointestinal lymphoma and malabsorption. We describe a patient from the United States with polypoid lymphoma of the gastrointestinal tract and a prolonged clinical course, who had the characteristic alpha heavy chain disease protein. He did not have malabsorption, and the gastrointestinal tract, though containing multiple discrete lymphomatous polyps had little plasmacytic reaction. Lymphosarcoma cell leukemia developed late in the course and the malignant cells had the characteristics of B lymphocytes but did not contain alpha heavy chains. The data support the contention that the lymphoma in this form of alpha heavy chain disease may involve B lymphocytes but that the abnormal protein may be produced by other cells. Polypoid varieties of gastrointestinal lymphoma in North America must now be considered part of the spectrum of alpha heavy chain disease and the diagnostic protein searched for diligently.

Full Text

Duke Authors

Cited Authors

  • Cohen, HJ; Gonzalvo, A; Krook, J; Thompson, TT; Kremer, WB

Published Date

  • March 1, 1978

Published In

Volume / Issue

  • 41 / 3

Start / End Page

  • 1161 - 1169

PubMed ID

  • 416900

International Standard Serial Number (ISSN)

  • 0008-543X

Digital Object Identifier (DOI)

  • 10.1002/1097-0142(197803)41:3<1161::aid-cncr2820410354>;2-j


  • eng

Conference Location

  • United States