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Hairy cell leukemia-associated familial lymphoproliferative disorder: immunologic abnormalities in unaffected family members.

Publication ,  Journal Article
Cohen, HJ; Shimm, D; Paris, SA; Buckley, CE; Kremer, WB
Published in: Ann Intern Med
February 1979

Three generations of a family were affected by hematologic malignancies: the proband had hairy cell leukemia, his brother chronic lymphocytic leukemia, his nephew cutaneous and central nervous system lymphoma, and his father chronic granulocytic leukemia. There was no clinical evidence for immune deficiency in 17 unaffected family members. Most family members had normal serum immunoglobulins and peripheral B-cells, but some had decreased T cells. Lymphocyte responses to phytohemagglutinin and concanavalin A were markedly reduced, while pokeweed mitogen responses were essentially normal. Delayed hypersensitivity responses determined by response to a 23-antigen skin test panel were markedly diminished, as were viral antibody titers. The decreased immune function in these family members suggests a role for the immune system in the emergence of hairy cell leukemia and other lymphoid malignancies, though further follow-up will be needed to see if other family members develop malignancies.

Duke Scholars

Published In

Ann Intern Med

DOI

ISSN

0003-4819

Publication Date

February 1979

Volume

90

Issue

2

Start / End Page

174 / 179

Location

United States

Related Subject Headings

  • T-Lymphocytes
  • Skin Neoplasms
  • Middle Aged
  • Male
  • Lymphoproliferative Disorders
  • Lymphoma
  • Leukocyte Count
  • Leukemia, Myeloid
  • Leukemia, Lymphoid
  • Leukemia, Hairy Cell
 

Citation

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Cohen, H. J., Shimm, D., Paris, S. A., Buckley, C. E., & Kremer, W. B. (1979). Hairy cell leukemia-associated familial lymphoproliferative disorder: immunologic abnormalities in unaffected family members. Ann Intern Med, 90(2), 174–179. https://doi.org/10.7326/0003-4819-90-2-174
Cohen, H. J., D. Shimm, S. A. Paris, C. E. Buckley, and W. B. Kremer. “Hairy cell leukemia-associated familial lymphoproliferative disorder: immunologic abnormalities in unaffected family members.Ann Intern Med 90, no. 2 (February 1979): 174–79. https://doi.org/10.7326/0003-4819-90-2-174.
Cohen HJ, Shimm D, Paris SA, Buckley CE, Kremer WB. Hairy cell leukemia-associated familial lymphoproliferative disorder: immunologic abnormalities in unaffected family members. Ann Intern Med. 1979 Feb;90(2):174–9.
Cohen, H. J., et al. “Hairy cell leukemia-associated familial lymphoproliferative disorder: immunologic abnormalities in unaffected family members.Ann Intern Med, vol. 90, no. 2, Feb. 1979, pp. 174–79. Pubmed, doi:10.7326/0003-4819-90-2-174.
Cohen HJ, Shimm D, Paris SA, Buckley CE, Kremer WB. Hairy cell leukemia-associated familial lymphoproliferative disorder: immunologic abnormalities in unaffected family members. Ann Intern Med. 1979 Feb;90(2):174–179.

Published In

Ann Intern Med

DOI

ISSN

0003-4819

Publication Date

February 1979

Volume

90

Issue

2

Start / End Page

174 / 179

Location

United States

Related Subject Headings

  • T-Lymphocytes
  • Skin Neoplasms
  • Middle Aged
  • Male
  • Lymphoproliferative Disorders
  • Lymphoma
  • Leukocyte Count
  • Leukemia, Myeloid
  • Leukemia, Lymphoid
  • Leukemia, Hairy Cell