Rituximab Therapy for HIV-Negative Multicentric Castleman’s Disease: Seven Years’ Experience at a Single Institute

Background: Multicentric Castleman’s disease (MCD) is an indolent lymphoproliferative disorder of unknown etiology, and is classified into three types: hyaline-vascular type, plasma-cell type, and mixed type. Recently, we reported the use of rituximab therapy for HIV-negative patients with MCD, after obtaining the informed consent of the patient and the approval of the Institutional Review Board (Br J Haematology 121, 818–819, 2003, Eur J Haematology 76. 119–123. 2006). Therefore, we tried to review the long-term follow-up (nine to 75 months) of all patients at a single institute.

Patients and Methods: Over seven years, 5 HIV-negative patients with MCD (3 men and 2 women; median age: 39, range: 26–61 years) were referred to Takamatsu Red Cross hospital. After clinical evaluation (chest and abdominal computed tomography scans, magnetic resonance imaging and Ga67 citrate scintigrams), lymphnode biopsy was performed for histological diagnosis. Rituximab was administered intravenously at a standard dose of 375 mg/m2 weekly, for 4 or 8 times without chemotherapy, and we analyzed the clinical course of MCD after rituximab therapy. During the follow-up, clinical symptoms were recorded. At a median follow-up of 49 months, biologic parameters (complete blood count, C-reactive protein, immunoglobulin G), and cytokines (soluble interleukin-2 receptor, Interleukin-6) were determined at different time points by standard procedures.

Results: Three of the five patients had histological evidence of hyaline-vascular type MCD, and two patients had plasma-cell type MCD. Two (both with hyaline vascular type) of the five MCD patients (40%) with MCD achieved almost complete remission with rituximab therapy when followed up for 50 to 75 months. Three (one with hyaline vascular type and two with plasma-cell type) of the five patients did not show clinical remission after riruximab administration.

Conclusion;Rituximab treatment provides a durable response in a some MCD (especially those with hyaline-vascular type) patients, but is not always successful.