NIH conference. Angioimmunoblastic lymphadenopathy with dysproteinemia.

A. D. Steinberg, Michael F Seldin, E. S. Jaffe, H. R. Smith, D. M. Klinman, A. M. Krieg, J. Cossman

Research output: Contribution to journalArticle

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Abstract

Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by a sudden onset of constitutional symptoms and lymphadenopathy. Patients often have hypergammaglobulinemia, autoantibodies, rashes, thrombocytopenia, or hemolytic anemia. Diagnosis requires a lymph node biopsy that shows architectural effacement, absence of germinal centers, arborization of postcapillary venules, and a polymorphous infiltrate that includes immunoblasts. Early in the disease, activated T cells in blood and lymph nodes stimulate B cells to proliferate and produce antibody. However, late in the disease, immune suppression may result from increased suppressor function. Clonal rearrangements, which are seen in all patients with regard to either the T-cell receptor beta-chain gene or immunoglobulin genes, have been followed by malignant transformation and frank lymphoma in some patients. Thus, this disorder stands partway between benign lymphoid proliferation and clonal lymphoid transformation. The prognosis of this disorder is poor; 75% of patients die within 2 years or develop a lymphoid malignancy. The rest usually go into a sustained remission. Current treatment with corticosteroid and immunosuppressive agents is unsatisfactory, especially because of late immunosuppression and predisposition to infections.

Original languageEnglish (US)
Pages (from-to)575-584
Number of pages10
JournalAnnals of Internal Medicine
Volume108
Issue number4
StatePublished - Apr 1988
Externally publishedYes

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Immunoblastic Lymphadenopathy
Lymph Nodes
T-Cell Receptor beta Genes
Hypergammaglobulinemia
Immunoglobulin Genes
Germinal Center
Venules
Hemolytic Anemia
Immune System Diseases
Immunosuppressive Agents
Exanthema
Thrombocytopenia
Autoantibodies
Immunosuppression
Lymphoma
Adrenal Cortex Hormones
B-Lymphocytes
T-Lymphocytes
Biopsy
Antibodies

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Steinberg, A. D., Seldin, M. F., Jaffe, E. S., Smith, H. R., Klinman, D. M., Krieg, A. M., & Cossman, J. (1988). NIH conference. Angioimmunoblastic lymphadenopathy with dysproteinemia. Annals of Internal Medicine, 108(4), 575-584.

NIH conference. Angioimmunoblastic lymphadenopathy with dysproteinemia. / Steinberg, A. D.; Seldin, Michael F; Jaffe, E. S.; Smith, H. R.; Klinman, D. M.; Krieg, A. M.; Cossman, J.

In: Annals of Internal Medicine, Vol. 108, No. 4, 04.1988, p. 575-584.

Research output: Contribution to journalArticle

Steinberg, AD, Seldin, MF, Jaffe, ES, Smith, HR, Klinman, DM, Krieg, AM & Cossman, J 1988, 'NIH conference. Angioimmunoblastic lymphadenopathy with dysproteinemia.', Annals of Internal Medicine, vol. 108, no. 4, pp. 575-584.
Steinberg AD, Seldin MF, Jaffe ES, Smith HR, Klinman DM, Krieg AM et al. NIH conference. Angioimmunoblastic lymphadenopathy with dysproteinemia. Annals of Internal Medicine. 1988 Apr;108(4):575-584.
Steinberg, A. D. ; Seldin, Michael F ; Jaffe, E. S. ; Smith, H. R. ; Klinman, D. M. ; Krieg, A. M. ; Cossman, J. / NIH conference. Angioimmunoblastic lymphadenopathy with dysproteinemia. In: Annals of Internal Medicine. 1988 ; Vol. 108, No. 4. pp. 575-584.
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