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The clinical behavior of localized and multicentric Castleman disease
J Herrada1, F Cabanillas, L Rice
1University of Texas M.D. Anderson Cancer Center, Baylor College of Medicine, and Methodist Hospital, Houston 77030, USA.
Insights
Castleman disease, a lymphoid proliferation, has localized and multicentric forms. Localized disease is curable with surgery, while multicentric Castleman disease requires chemotherapy or prednisone for complete remission.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Castleman disease is a rare condition characterized by excessive lymphoid tissue proliferation.
- It presents as localized or multicentric clinical types with hyaline vascular, plasma-cell, or mixed histologic variants.
Purpose of the Study:
- To investigate the clinical characteristics, treatment strategies, and outcomes for patients diagnosed with Castleman disease.
- To differentiate between localized and multicentric Castleman disease presentations and their respective management approaches.
Main Methods:
- A retrospective case series analysis of 15 patients diagnosed with Castleman disease between 1977 and 1995.
- Patients were classified by disease type (localized vs. multicentric) and histologic variant, and treatment outcomes were recorded.
Main Results:
- All 7 patients with localized Castleman disease treated with surgical excision were disease-free post-treatment.
- For multicentric Castleman disease, 3 patients receiving combination chemotherapy achieved disease-free status.
- Prednisone treatment resulted in survival but required ongoing therapy for relapses, while surgery alone for multicentric disease led to mortality.
Conclusions:
- Localized and multicentric Castleman disease represent distinct clinical entities despite overlapping histology.
- Complete surgical resection offers a cure for localized Castleman disease.
- Effective management of multicentric Castleman disease necessitates timely chemotherapy or prednisone administration for complete remission.
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.
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