Page 186 - Haematologica Vol. 109 - July 2024

ARTICLE - Disease burden of idiopathic MCD
M. Sarmiento Bustamante et al.
these clinical signs, we found a substantial subset of NOS patients with the same severe clinical features. Among the 102 patients, 77 (76%) presented with severe disease at diagnosis, including 57 (93%) TAFRO patients and 20 (51%) NOS patients (Table 1). These data demonstrate that iMCD patients of any age and subtype can have severe and burdensome disease.
Idiopathic multicentric Castleman disease patients with TAFRO face significant hospitalization burden
We examined the number of hospitalized days due to iMCD symptoms as another measure of disease burden. In the year surrounding diagnosis (6 months preceding and after the diagnostic biopsy), iMCD patients were hospitalized for a median (IQR) of 18 (0.5-38.0) days. When we stratified
by days hospitalized prior to and following diagnosis, we found that patients were hospitalized a median (IQR) of 5 (0-13.8) days in the 6 months preceding their diagnosis (Figure 2A). In the 6 months following diagnosis, patients were hospitalized a median (IQR) of 7.5 (0-25.0) days. Eleven patients (10.8%) spent more than 60 days in hospital in the year surrounding their diagnosis, highlighting the morbidity of this disease.
Next, we examined hospitalizations by subtype of iMCD to evaluate any differences in burden of hospitalizations. We found that TAFRO patients spent significantly more days in hospital than did NOS patients. The median (IQR) days in hospital in the year surrounding diagnosis was 36 (18- 61) for patients with TAFRO and 0 (0-4) for NOS patients (W=153.5, P=1.3x10-13) (Figure 2B). In order to understand
A
B
Figure 2. Patients with idiopathic multicentric Castleman disease face a high burden of hospitalizations. (A) Patients with idiopathic multicentric Castleman disease (iMCD) are hospitalized a large proportion of time in the 6 months leading up to their diag- nosis and the 6 months following diagnosis (year around diagnosis). The graph depicts each patient on the Y axis with the time hospitalized prior to diagnosis and the number of days hospitalized after diagnosis. Red indicates the time that iMCD pa- tients with TAFRO (thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly) spent in hospital, and blue indicates the time that patients with iMCD not otherwise specified (NOS) were hospitalized. In the 6 months prior to diagnosis, patients were hospitalized a mean (standard deviation [SD]) of 10.6 (17.5) days and a median (interquartile range [IQR]) of 5 (0-13.8) days, while in the year following diagnosis, patients were hospitalized a mean (SD) of 18.9 (28.2) days and a median (IQR) of 7.5 (0-25.0) days. (B) A sample of the USA population from the 2018 National Health Information Survey (NHIS), a proxy for the general population, spent significantly less time hospitalized in a 12-month period (median [IQR]: 0 [0-0], mean [SD]: 0.24 [1.3] days) than either TAFRO patients (me- dian [IQR]: 35 [18-61], mean [SD]: 46.0 [42.0] days; P<2.2x10-16) or NOS patients (me- dian [IQR]: 0 [0-4], mean [SD]:4.9 [9.0] days; P=8.3x10-13) spent hospitalized in the year around diagnosis. TAFRO patients were hospitalized significantly more days than NOS patients within the year of diagnosis (P=1.3x10-13). ***P<0.001.
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