Castleman disease is a non-clonal lymphoproliferative disorder with 2 clinical (unicentric, multicentric) and 4 histomorphological (hyaline vascular, plasma cell, mixed, plasmablastic) forms which combine creating a pleomorphic picture of this rare entity. In our work, the largest documented cohort in the Czech Republic was analyzed focusing on diagnostics and particularly on therapy.
The retrospective study (1998-2013) included 10 patients, 6 males, 4 females. Patients with unicentric form (3) underwent surgical sanitation. Patients with multicentric form (7) were followed-up only (2) or extirpation of the largest mass was carried out (1) or a systemic therapy was administered (4) which comprised the following regimens: R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), CTD/CAD/CVD (cyclophosphamide, thalidomide/adriamycin/bortezomib, dexamethasone), further including monotherapies with tocilizumab, thalidomide and lenalidomide and in one case (associated POEMS syndrome, i.e. polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) autologous stem cell transplantation after melphalan conditioning was performed. During treatment response monitoring, all patients underwent PET/CT examination (fluorodeoxyglucose positron emission tomography/computed tomography).
The remission rate was 50% (3 unicentric forms with remission lasting 51, 8 and 9 months, resp.; 2 multicentric forms with remission lasting 3 months during thalidomide therapy and 12 months after lenalidomide therapy), stable disease was observed in 40% of cases (multicentric forms, 2 without any treatment followed-up for 171 and 24 months, resp.; 1 after systemic therapy followed-up for 23 months; 1 after two extirpations with stable lymphadenopathy for 15 years, where the first operation was 27 years ago). In one patient (10%), the associated POEMS syndrome progressed rapidly with fatal consequences (4 months follow-up).
Unlike unicentric forms completely curable by excision, multicentric forms are often treatment-refractory. Concerning high cost-effectiveness, good tolerability and documented efficacy also in rituximab-resistant cases, we prefer immunomodulatory drugs (particularly thalidomide) for managing multicentric Castleman disease in our center.