Clinical features and outcome of patients with HIV-negative multicentric Castleman’s disease treated with combination chemotherapy: a report on 10 patients

To investigate the clinical characteristics and outcome of patients with HIV-negative multicentric Castleman’s disease (MCD) treated exclusively with combination chemotherapy, and review literature to improve the diagnosis and management of this disease. A retrospective study was performed on the medical records of 10 patients with HIV-negative MCD treated exclusively with combination chemotherapy at one medical institution from May 2004 to April 2012. And relevant clinical, pathological, radiographic, and laboratory data were examined in order to evaluate treatment responses, with symptom onsets and survival period serving as the endpoints of the assessment. All patients have multifocal lymphadenopathy, and the associated system symptoms are found in 80 % of the cases. All patients were treated with lymphoma-based chemotherapy alone. The duration of follow-up ranged from 5 to 77 months for nine patients. Four patients were treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) alone: One was alive with no evidence of disease, and three were alive with disease. Three patients received cyclophosphamide, vincristine, and prednisone (COP) alone: One remained alive with disease, and two experienced recurrences and passed away. Two had only minimal response to COP and were switched to CHOP, and they were still alive with disease. MCD is a more progressive clinical entity, and long-term follow-up is necessary. CHOP chemotherapy may be an effective treatment option for patients with MCD, whereas when to start chemotherapy, how many cycles of chemotherapy required, and the role of combined radiotherapy remain to be further studied.