Multicentric angiofollicular lymph node hyperplasia with peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis in women. A distinct syndrome

J. M. Feigert, D. L. Sweet, M. Coleman, D. Variakojis, N. Wisch, J. Schulman, M. H. Markowitz

Research output: Contribution to journalArticlepeer-review

50 Scopus citations

Abstract

Four women with multicentric angiofollicular lymph node hyperplasia had a distinct clinical syndrome characterized by peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis. The nodes displayed typical morphologic changes of the plasma cell variant of multicentric angiofollicular lymph node hyperplasia. The pathologic changes are morphologically distinct from angioimmunoblastic lymphadenopathy with dysproteinemia although clinical similarities do exist. In these four cases, the lymphadenopathy was usually bulky and multicentric. There was frequent splenic involvement. The neuropathies were severe and disabling. Clinical courses have been variable with some responses to therapy with steroids and alkylating agents. No neoplastic transformations have occurred. Multicentric angiofollicular lymph node hyperplasia may represent a reactive lesion in which the antigenic stimulus is unknown but results in follicular hyperplasia, angiogenesis, and the systemic manifestations of hyperimmune stimulation. We believe this clinical syndrome may represent a distinct variant of multicentric angiofollicular lymph node hyperplasia, and it requires close observation for neoplastic transformation and other complications of its multisystem nature.

Original languageEnglish
Pages (from-to)362-367
Number of pages6
JournalAnnals of Internal Medicine
Volume113
Issue number5
DOIs
StatePublished - 1990

Keywords

  • Giant lymph node hyperplasia
  • IgA dysproteinemia
  • peripheral nerve diseases
  • pseudotumor cerebri
  • thrombocytosis

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