CLINICAL FEATURES AND PROGNOSIS OF MULTICENTRIC CASTLEMAN's DISEASE

Respirology ◽  
2017 ◽  
Vol 22 ◽  
pp. 258-258
Keyword(s):  
Clinical Features ◽  
Castleman’S Disease ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
Multicentric Castleman's Disease

Clinical Features and Outcome in HIV-Associated Multicentric Castleman's Disease

2011 ◽  
Vol 29 (18) ◽  
pp. 2481-2486 ◽  
Author(s):  
Mark Bower ◽  
Tom Newsom-Davis ◽  
Kikkeri Naresh ◽  
Shairoz Merchant ◽  
Belinda Lee ◽  
...  
Keyword(s):  
Overall Survival ◽  
Clinical Features ◽  
Survival Rates ◽  
Castleman’S Disease ◽  
Progression Free Survival ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
Clinical Criteria ◽  
Time To Relapse ◽  
Multicentric Castleman's Disease

Purpose To describe clinical features, treatment outcomes and relapse rates in HIV-associated multicentric Castleman's disease (MCD) in a sizeable mature cohort. Methods From a prospective database, we identified 61 HIV-seropositive patients with histologically confirmed MCD (median follow-up, 4.2 years). Since 2003, 49 patients with newly diagnosed MCD have been treated with rituximab with (n = 14) or without (n = 35) etoposide. Results At MCD diagnosis, 55 (90%) of 61 patients met proposed clinical criteria defining an attack. Four patients (7%) had histologic evidence of coexisting lymphoma, and one developed lymphoma 2 years after treatment. The incidence of lymphoma is 28 per 1,000 patient years. With rituximab-based treatment, the overall survival was 94% (95% CI, 87% to 100%) at 2 years and was 90% (95% CI, 81% to 100%) at 5 years compared with 42% (95% CI, 14% to 70%) and 33% (95% CI, 6% to 60%) in 12 patients treated before introduction of rituximab (log-rank P < .001). Four of 49 rituximab-treated patients have died; three died as a result of MCD within 10 days of diagnosis, and one died as a result of lymphoma in remission of MCD. Eight of 46 patients who achieved clinical remission suffered symptomatic, histologically confirmed MCD relapse. The median time to relapse was 2 years, and all have been successfully re-treated and are alive in remission. The 2- and 5-year progression-free survival rates for all 49 patients treated with rituximab-based therapy were 85% (95% CI, 74% to 95%) and 61% (95% CI, 40% to 82%), respectively. Conclusion HIV-associated MCD is a remitting-relapsing disease. The outlook has improved dramatically in recent years with the introduction of rituximab-based therapy and yields high overall survival rates.

scholarly journals Membranous nephropathy associated with multicentric Castleman’s disease that was successfully treated with tocilizumab: a case report and review of the literature

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ryosuke Saiki ◽  
Kan Katayama ◽  
Yosuke Hirabayashi ◽  
Keiko Oda ◽  
Mika Fujimoto ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Periodic Acid ◽  
Castleman’S Disease ◽  
Basement Membranes ◽  
The Body ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
High Zinc ◽  
Secondary Membranous Nephropathy ◽  
Multicentric Castleman's Disease

Abstract Background Multicentric Castleman’s disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6. Although renal complications of Castleman’s disease include AA amyloidosis, thrombotic microangiopathy, and membranoproliferative glomerulonephritis, membranous nephropathy is relatively rare. We experienced a case of secondary membranous nephropathy associated with Castleman’s disease. Case presentation The patient was a 43-year-old Japanese man who had shown a high zinc sulfate value in turbidity test, polyclonal hypergammaglobulinemia, anemia, and proteinuria. A physical examination revealed diffuse lymphadenopathy, an enlarged spleen and papulae of the body trunk. A skin biopsy of a papule on the patient’s back showed plasma cells in the perivascular area and he was diagnosed with multicentric Castleman’s disease, plasma cell variant. Kidney biopsy showed the appearance of bubbling in the glomerular basement membranes in Periodic acid methenamine silver stain and electron microscopy revealed electron dense deposits within and outside the glomerular basement membranes. Since immunofluorescence study showed predominant granular deposition of IgG1 and IgG2, he was diagnosed with secondary membranous nephropathy associated with Castleman’s disease. He was initially treated with prednisolone alone, however his biochemical abnormalities did not improve. After intravenous tocilizumab (700 mg every 2 weeks) was started, his C-reactive protein elevation, anemia, and polyclonal gammopathy improved. Furthermore, his urinary protein level declined from 1.58 g/gCr to 0.13 g/gCr. The prednisolone dose was gradually tapered, then discontinued. He has been stable without a recurrence of proteinuria for more than 6 months. Conclusions Tocilizumab might be a treatment option for secondary membranous nephropathy associated with Castleman’s disease.

Sign in / Sign up

Export Citation Format

Share Document