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ICDO
International
Castlemans
Disease
Organization



Mayo Clin Proc 1995 Oct;70(10):969-977

Castleman's disease.

Shahidi H, Myers JL, Kvale PA

OBJECTIVE: To describe the two variants of Castleman's disease--the hyaline-vascular type and the plasma-cell type--and discuss the associated histologic features. DESIGN: We present a case of the hyaline-vascular type and review the literature. RESULTS: Castleman's disease was once thought to be localized and self-limited, but in recent years, reports have described a multicentric variety with severe systemic manifestations and, at times, an inexorable clinical course. Unlike the localized type for which surgical excision is curative regardless of the histologic type, multicentric disease often necessitates aggressive systemic therapy and portends a poor outcome. Little is known about the cause of this disorder, but the bulk of evidence points toward faulty immunoregulation that results in excessive proliferation of B lymphocytes and plasma cells in lymphoid organs. CONCLUSION: Castleman's disease is rare and poorly understood. The diagnosis is "contextual" and must be considered in the appropriate clinical setting and only after all other causes of lymphadenopathy have been investigated and excluded. The optimal therapeutic regimen is unknown.


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DISCLAIMER:

CASTLEMAN's DISEASE HOMEPAGE is provided for educational purposes only. Consult your own physician regarding the applicability of any opinions or recommendations with respect to your symptoms or medical condition.

* CASTLEMAN's DISEASE HOMEPAGE is intended as a reference for health care professionals. The authors and editors have used sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication.

Medical knowledge changes rapidly. In view of the possibility of human error or changes in medical science, neither the authors, the editors, St. Vincent Hospital, nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they are not responsible for any errors or omissions or for the results obtained from the use of such information. Readers are encouraged to confirm the information contained herein with other sources.


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