CDCN team members have recently co-authored four papers in highly regarded international journals, including two this week in the Lancet Hematology. One Lancet Hematology paper, led by Amy Liu and Dr. David Fajgenbaum, is the largest and most in-depth study of HHV-8-negative or "idiopathic (meaning unknown cause)" multicentric Castleman Disease (iMCD)..This study summarized data from 255 cases of Castleman Disease.
Findings from the study:
- Patients with Castleman Disease are three times more likely to have cancer than people in the same age group.
- Identified effective treatment options that have been used in Castleman Disease
- Showed that Castleman Disease can be deadly and that patients have frequent relapses.
- (click here for the article).
The other article in the Lancet Hematology, written by Drs. David Fajgenbaum, Jason Ruth, Dermot Kelleher, and Arthur Rubinstein, describes the many hurdles in rare disease research. It also describes the CDCN's innovative "Collaborative network approach." This new plan provides a blueprint for accelerating research for rare diseases.
Drs. Chris Nabel and Fajgenbaum also co-authored with over a dozen Japanese researchers the largest-ever case series of HHV-8-negative MCD patients with the newly-described "TAFRO syndrome" in the American Journal of Hematology. Patients with TAFRO syndrome have a low platelet count, fluid accumulation, fevers, fibrosis (thickening or scarring) in bone marrow, and enlarged liver and spleen. This study included the first reported case in the USA, but it is clear that there are many more cases of TAFRO throughout the USA and world that have not been reported in the literature (click here for the article).
Drs. Fajgenbaum and Razelle Kurzrock also published a review of siltuximab, which is the only US Food and Drug Administration (FDA)- and European Medicines Agency (EMA)- approved therapy for HHV-8-negative MCD, in the Journal of Immunotherapy (click here for the article).
Find other key CD publications by clicking here.