Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Castleman disease (CD) is a group of lymphoproliferative disorders with shared histopathological traits.
• CD presents in unicentric (UCD) and multicentric (MCD) forms, with MCD further classified into human herpesvirus-8 (HHV-8) associated and idiopathic (iMCD) subtypes.

Purpose of the Study:

• To compare the clinical characteristics, demographics, and outcomes of different Castleman disease subtypes.
• To elucidate the distinct epidemiological and prognostic profiles of UCD, HHV-8-associated MCD, and iMCD.

Main Methods:

• An observational study was conducted on 53 patients diagnosed with Castleman disease.
• Data collected included patient demographics, disease subtype (UCD, HHV-8 MCD, iMCD), and clinical outcomes such as relapses and mortality.

Main Results:

• A total of 53 CD patients were analyzed: 20 UCD, 33 MCD (10 HHV-8-associated, 23 iMCD).
• Median age was lower in UCD (30 years) versus iMCD (49 years; p=0.004).
• Males predominated in HHV-8 MCD (100%), females in UCD (75%). Relapses were more frequent in iMCD (57%) vs. UCD (10%; p=0.002). Mortality was significantly higher in iMCD and HHV-8 MCD compared to UCD.

Conclusions:

• Unicentric Castleman disease (UCD) is characterized by younger age of onset and female predominance, presenting as a benign condition.
• Idiopathic multicentric Castleman disease (iMCD) represents a distinct entity with a higher propensity for disease relapse and increased mortality.
• These findings highlight the need for subtype-specific management strategies in Castleman disease.