Wednesday, January 14, 2015

Castleman disease

for Castleman disease
Hepcidin, Arlet at al" The results of this unique case study (1) explain the mechanism of iron deficiency observed in some children with CD, (2) confirm in vivo the regulatory effect of IL-6 in human hepcidin production, and (3) suggest that iron deficiency is a causal link between IL-6 and anemia of chronic disease."
from conference correspodent:
Siltuximab Efficacy in Multicentric Castleman's Disease Is Independent of Baseline Symptom Burden
Multicentric Castleman's Disease (MCD) is a rare, highly symptomatic, systemic lymphoproliferative disorder driven by dysregulated interleukin (IL)-6 signaling

from Johnson :"
Fifty-three patients were randomized to the SYLVANT arm at a dose of 11 mg/kg and 26 patients were randomized to the placebo arm. Patients had symptomatic MCD and were HIV negative and HHV-8 negative.[1] In this pivotal study, which led to the FDA approval, more than one-third of patients in the SYLVANT arm had a durable tumor and symptomatic response to treatment plus best supportive care (BSC), compared to none of the patients who received placebo plus BSC (34 percent versus 0 percent; 95 percent CI: 11.1, 54.8; p=0.0012). A durable response was defined as tumor and symptomatic response (reduction in tumor size and disease symptoms) that persisted for a minimum of 18 weeks without treatment failure."
The FDA approved the drug based on these findings

A trial of Dexamethasone and Rituxan could also be given....
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