Page 189 - Haematologica Vol. 109 - July 2024

ARTICLE - Disease burden of idiopathic MCD M. Sarmiento Bustamante et al. AB
Figure 4. Patients with idiopathic multicentric Castleman disease have numerous comorbid and morbid conditions contributing to the burden of the disease. (A) Prior to diagnosis, the most commonly diagnosed comorbidities among the full cohort of patients with idiopathic multicentric Castleman disease (iMCD) mirrored common comorbidities among the USA population and included hypertension (N=26, 25.5%), obesity (N=23, 22.5%), asthma (N=21, 20.6%), gastroesophageal reflux disease (N=14, 13.7%), and de- pression (N=11, 10.8%). (B) Following the diagnosis of iMCD, patients experienced an array of burdensome comorbidities and mor- bidities including acute renal failure (N=49, 48.0%), chronic kidney disease/chronic renal insufficiency (N=16, 15.7%) and iron de- ficiency anemia (N=11, 10.8%) among others. GERD: gastroesophageal reflux disease; ADHD: attention-deficit hyperactivity disorder; CKD/CRI: chronic kidney disease/chronic renal insufficiency; TMA: thrombotic microangiopathy; OSA: obstructive sleep apnea; NOS: not otherwise specified; TAFRO: thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/ renal failure, and organomegaly.
with 0 being the worst health imaginable and 100 being the best health imaginable. This compares to a mean score of 80.4 in the general USA population.21 That same day, the patients reported a median MCD symptom score of 29.5 (IQR, 20-37; min-max: 16-96) with 16 being the best pos- sible score (no iMCD symptoms) and 96 being the worst possible score. We investigated whether there was any relationship between a patient’s QOL score and number of days hospitalized prior to completing the survey, but did not found a correlation (R=0.038; P=0.8) (Online Sup- plementary Figure S3). We also looked for differences in subsequent QOL scores between patients who presented with severe disease and mild/moderate disease at baseline as well as between TAFRO and non-TAFRO patients and found no significant differences between groups (TAFRO vs. NOS: W=481, P=0.31; severe vs. mild/moderate at baseline: W=298.5, P=0.61). We determined the correlation between QOL score and MCD symptom score based on data col- lected a median (IQR) of 3.9 (2.3-8.0) years after diagnosis. QOL scores were inversely correlated with MCD symptom score (R=-0.69; P<0.001) (Figure 5). This negative correlation appeared to hold for patients with both NOS and TAFRO subtypes. This suggests that several years after diagnosis, patients reporting active iMCD symptoms may be having a decrease in their QOL from those symptoms.
Figure 5. Quality of life of patients with idiopathic multicentric Castleman disease is inversely correlated with degree of symptoms. There is a correlation between quality-of-life score and idiopathic multicentric Castleman disease (iMCD) symp- tom score. Lower quality of life correlates with higher iMCD symptom score and higher quality of life correlates with low- er iMCD symptom score (R=-0.69, P<0.001). NOS: not otherwise specified; TAFRO: thrombocytopenia, anasarca, fever/elevat- ed C-reactive protein, reticulin fibrosis/renal failure, and organomegaly.
Haematologica | 109 July 2024
2203