The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry

04/12/2022  Castleman disease is a group of rare disorders, and idiopathic multicentric Castleman disease (iMCD) is the most severe subtype characterized by enlarged lymph nodes in multiple regions in the body, inflammatory symptoms, and life-threatening organ dysfunction. The estimated five-year overall survival rate varies considerably, ranging from 50-77%. Using our natural history registry, ACCELERATE (, we were able to compare fatal and non-fatal outcomes in iMCD to identify laboratory features that may be clues that patients are at increased risk of death. We found that low IgM and platelet counts around the time of diagnosis were found more often in patients who died from their disease compared to those who go on to survive. These abnormal lab tests may indicate immune dysregulation or an increased risk of bleeding events. More research is needed to improve treatments and prevent CD patients from dying. Check out results from this study here!

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Castleman disease (CD) describes a group of rare, potentially fatal lymphoprolifera- tive disorders. To determine factors associated with mortality in CD, we analysed data from deceased patients in the ACCELERATE registry and compared them with matched controls. We analysed demographic, treatment and laboratory data from all deceased CD patients, matched controls and a subgroup of idiopathic multicentric Castleman disease (iMCD) patients. Of the 140 patients in ACCELERATE with a confirmed CD diagnosis, 10 had died. There were 72 patients with confirmed iMCD; six were deceased. The deceased CD cohort had more hospitalisations per year, higher overall hospitalisations and more days hospitalised per month, and received more treatment regimens per year than the matched-control group. Analysis of labo- ratory values showed a significantly decreased absolute lymphocyte count at months 3 and 6 in the deceased cohort compared with controls. Among iMCD patients, there was a higher proportion of iMCD-TAFRO (thrombocytopenia, anasarca, fever, re- ticulin myelofibrosis, renal dysfunction and organomegaly) cases in the deceased group. The deceased iMCD group had significantly lower immunoglobulin M, inter- national normalised ratio and platelet count. These data demonstrate that there may be differences between patients who have fatal and non-fatal outcomes, and provide preliminary suggestions for parameters to evaluate further.

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