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The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry
Author(s) -
Fajgenbaum David C.,
Pierson Sheila K.,
Kanhai Karan,
Bagg Adam,
Alapat Daisy,
Lim Megan S.,
Lechowicz Mary Jo,
Srkalovic Gordan,
Uldrick Thomas S.,
van Rhee Frits
Publication year - 2022
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.18214
Subject(s) - medicine , cohort , myelofibrosis , organomegaly , hematology , castleman disease , disease , gastroenterology , surgery , bone marrow
Summary Castleman disease (CD) describes a group of rare, potentially fatal lymphoproliferative 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 laboratory 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, reticulin myelofibrosis, renal dysfunction and organomegaly) cases in the deceased group. The deceased iMCD group had significantly lower immunoglobulin M, international 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.