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ICDO
International
Castlemans
Disease
Organization

The clinical behavior of localized and multicentric Castleman disease.

Herrada J, Cabanillas F, Rice L, Manning J, Pugh W

University of Texas M.D. Anderson Cancer Center, Baylor College of Medicine, and Methodist Hospital, Houston 77030, USA.

BACKGROUND: Castleman disease, an unusual condition of unknown cause consisting of a massive proliferation of lymphoid tissue, remains a clinicopathologic diagnosis. Three histologic variants (hyaline vascular, plasma-cell, and mixed) and two clinical types (localized and multicentric) of Castleman disease have been described. OBJECTIVE: To analyze the clinical features, management, and outcome of patients with Castleman disease. DESIGN: Case series. SETTING: University referral hospitals. PATIENTS: All patients with Castleman disease who were seen at Texas Medical Center, Houston, Texas, between 1977 and 1995. INTERVENTIONS: Surgical excision for localized disease; surgery, combination chemotherapy, or prednisone for multicentric disease. MEASUREMENTS: Patients were identified according to initial presentation as having localized or multicentric Castleman disease. Patients within each group were further subdivided according to whether they had hyaline vascular, plasma-cell, or mixed disease. RESULTS: Data from 15 patients were analyzed. All 7 patients with localized disease underwent surgical excision and remain free of disease. The 8 patients with multicentric disease were further subdivided according to initial treatment: Three patients who received combination chemotherapy are currently alive and free of disease; 2 patients treated with prednisone are alive but have needed intermittent maintenance therapy for disease reactivations; and 2 patients treated with surgery only have died, 1 of infectious complications and 1 of non-Hodgkin lymphoma. CONCLUSIONS: Localized and multicentric Castleman disease are different clinical disorders with overlapping histologic features. Localized disease can be cured with surgery, but complete remissions in patients with multicentric disease have been achieved only with chemotherapy or prednisone given at the time of diagnosis.

Ann Intern Med 1998 Apr 15;128(8):657-62

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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.