Page 188 - Haematologica Vol. 109 - July 2024

ARTICLE - Disease burden of idiopathic MCD
cohort following iMCD onset by organ systems, revealing other serious conditions such as atypical hemolytic uremic syndrome (n=5, 4.9%), acute cholecystitis (n=5, 4.9%), and congestive heart failure (n=4, 3.9%) (Online Supplementary Table S7). Few malignancies were identified following diag- nosis; the most common malignancy was papillary thyroid carcinoma, which occurred in two patients 1.3 and 3.6 years after iMCD diagnosis (Online Supplementary Table S8).
M. Sarmiento Bustamante et al. Quality of life is inversely correlated with symptoms in
idiopathic multicentric Castleman disease
We questioned patients about their experiences living with iMCD by asking about QOL and the presence of on- going iMCD-related symptoms. Fifty-nine (57.8%) patients responded. At a median (IQR) of 3.9 (2.3-8.0) years after diagnosis, iMCD patients reported a median (IQR) QOL score of 80 (71.5-90.0) in the EQ-5D-5L, on a scale of 0 to 100
AB
D
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Figure 3. Patients with idiopathic multicentric Castleman disease experience a range of organ system involvement, require var- ious hospital interventions, and demonstrate ongoing flares. (A) Organ system involvement in idiopathic multicentric Castleman disease (iMCD) ranked from most frequently observed to least frequently observed. Over 97% of the iMCD cohort experienced some hematopoietic dysfunction. Notably, both patients with TAFRO (thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly) and those with iMCD not otherwise specified (NOS) experienced sig- nificant organ system involvement and dysfunction. (B) The severity of organ dysfunction is reflected by the degree of healthcare intervention(s) required. Over one-quarter of patients (N=27 [26.5%]) required the use of a ventilator, and 17 (16.7%) patients re- quired dialysis. Additionally, 47 (46.0%) patients required fluid removal (paracentesis), 42 (41.1%) patients received a red blood cell transfusion, and 22 (21.6%) patients received a platelet transfusion. (C) NOS patients spent a significantly greater proportion of time in flare from presentation until last known information (median [interquartile range]: 52.3% [21.0-99.6]) compared to TAFRO patients (18.9% [10.8-52.5], W=1673; P=0.004). (D) Each patient is represented by a vertical bar. The bar extends the length of follow-up from the start of the first flare. NOS patients are represented on the left, and TAFRO patients are represented on the right. The blue bars represent the proportion of time NOS patients spent in flare. The red bars represent the proportion of time TAFRO patients spent in flare. The designation ‘d’ indicates a deceased patient. RBC: red blood cells.
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