Efficacy of rituximab in an aggressive form of multicentric Castleman disease associated with immune phenomena

Abstract

Multicentric Castleman disease (MCD) is an uncommon lymphoproliferative disorder for which the best therapeutic option is not yet well established. Immune-related disorders are rare complications of MCD. We report on an MCD case in a 23-year-old patient with extensive abdominal involvement and associated immune hemolytic anemia and Raynaud phenomenon. He was negative for human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8). After 8 courses of the anti-CD20 monoclonal antibody (rituximab), the patient achieved complete remission. Interestingly, Raynaud phenomenon disappeared under treatment and no new hemolytic events occurred. Anti-CD20 antibody treatment could be an attractive therapeutic approach for MCD, mainly when immune-related disorders are associated. Am.

Doença de Castleman associada a sarcoma de células dendríticas foliculares e miastenia gravis

“…(19) Treatments with corticosteroids, immunomodulators, and monoclonal antibodies have been used, mainly for the control of any accompanying immunological disorders, because surgery does not affect the prognosis. (19,20) In the case reported here, we call attention to the coexistence of multiple diseases, a fact that might hinder the diagnosis, because, in this patient, the initial diagnosis was lung neoplasm associated with the Eaton-Lambert syndrome. However, the histopathological and immunohistochemical findings of the resected specimen, in addition to confirming the hypothesis of Castleman's disease, indicated a rare form of degeneration to follicular dendritic cell sarcoma.…”

Castleman's Disease: From Basic Mechanisms to Molecular Therapeutics

“…Clinical response was associated with a decrease in viral load. Rituximab has also been used successfully in other circumstances, such as in UCD [68], MCD associated with autoimmune phenomenon [69], and POEMS [70].…”

Efficacy of bortezomib in refractory form of multicentric Castleman disease associated to poems syndrome (MCD-POEMS variant)

“…Chemotherapy (CHOP or CVAD) has shown response rates around 90%, with 50% showing complete response [5]. In our patient's case, only a partial response was achieved.…”

“…Although therapeutic efficacy of antibody anti-IL6 receptor and anti-IL6 were reported [6,7], we did not have access to these drugs. Positive outcomes have been published regarding treatment with the anti-CD20 drug Rituximab [5]; in case of our patient, no response was obtained. Other therapeutic approaches such as the antiviral agents yielded nondurable response or no improvement [8,9].…”

“…Steroids can control inflammation [2] and improve response rates. Although these effects may be transient, they appear in about 60% of patients [5]. Interferon-alpha (INF-alpha) has both immunoregulatory and antiviral properties, making it a potentially effective therapy for patients with MCD [2].…”