Overview of Castleman disease

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Castleman disease (CD) is a complex group of disorders characterized by distinct histopathological features but diverse etiologies, clinical presentations, and prognoses.
• CD includes unicentric CD (UCD) and multicentric CD (MCD), with MCD further classified into idiopathic MCD (iMCD), HHV-8 associated MCD (HHV8-MCD), and POEMS-associated MCD (POEMS-MCD).
• Idiopathic MCD (iMCD) has specific subtypes, including iMCD-TAFRO and iMCD-NOS.

Purpose of the Study:

• To summarize recent advancements in the diagnosis, classification, pathogenesis, and therapy of Castleman disease.
• To highlight the impact of new treatments on patient outcomes.
• To emphasize the role of collaborative networks and patient registries in driving research progress.

Main Methods:

• Review of recent scientific literature and clinical data on Castleman disease.
• Analysis of treatment outcomes for different subtypes of CD, including HHV8-MCD and iMCD.
• Description of the Castleman Disease Collaborative Network (CDCN) initiatives and the ACCELERATE registry.

Main Results:

• Effective retroviral therapy and rituximab have improved outcomes for HHV8-MCD.
• Anti-interleukin-6 therapies show high efficacy in many iMCD patients, though refractory cases require further investigation.
• Significant progress in understanding and managing CD has been achieved through collaborative efforts.

Conclusions:

• Castleman disease management has seen substantial progress, with improved therapies for specific subtypes.
• Continued collaboration among researchers, clinicians, and patients, alongside registry participation, is crucial for future breakthroughs.
• The development of targeted therapies and the establishment of research networks are transforming the landscape of Castleman disease care.