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Multicentric Angiofollicular Lymph Node Hyperplasia with Peripheral Neuropathy, Pseudotumor Cerebri, IgA Dysproteinemia, and Thrombocytosis in Women: A Distinct Syndrome

John M. Feigert, MD; Donald L. Sweet, MD; Morton Coleman, MD; Diana Variakojis, MD; Nathaniel Wisch, MD; Julius Schulman, MD; and Martin H. Markowitz, MD
[+] Article, Author, and Disclosure Information

Requests for Reprints: Morton Coleman, MD, 407 East 70th Street, New York, NY 10021.

Current Author Addresses: Dr. Feigert: New Hampshire Oncology-Hematology, PA, 200 Technology Drive, Hooksett, NH 03106.

Dr. Sweet: 333 Chestnut Street, Suite LL3, Hinsdale, IL 60521.

Dr. Coleman: 407 E. 70th Street, New York, NY 10021.

Dr. Variakojis: Department of Pathology, Northwestern University Medical College, Ward Memorial Building, 303 E. Chicago Avenue, Chicago, IL 60611.

Drs. Wisch and Schulman: 12 East 86th Street, New York, NY 10028.

Dr. Markowitz: 250 East 73rd Street, New York, NY 10021.

©1990 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1990;113(5):362-367. doi:10.7326/0003-4819-113-5-362
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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.





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