Non-Clonal Lymphoproliferative Diseases

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Castleman disease encompasses a poorly understood spectrum of disorders.
• Recent advancements, particularly from the Castleman's Disease Collaborative Network, are enhancing classification and understanding.
• Accurate classification is crucial for comprehending underlying pathology and optimizing patient care.

Purpose of the Study:

• To outline the current understanding and classification of Castleman disease.
• To highlight the importance of recognizing and correctly categorizing Castleman disease based on established criteria.
• To review emerging therapeutic strategies for different Castleman disease subtypes.

Main Methods:

• Classification by anatomic spread (unicentric, oligocentric, multicentric).
• Histopathological analysis (hypervascular to plasmacytic spectrum).
• Clinical phenotype assessment (NOS, TAFRO, IPL, HHV8-associated, POEMS-associated).

Main Results:

• Castleman disease requires classification by anatomy, histopathology, and clinical phenotype.
• Therapeutic approaches are evolving, moving beyond retrospective data.
• Unicentric disease is primarily treated with surgery.

Conclusions:

• Multicentric Castleman disease treatment is subtype-specific.
• Interleukin-6 blockade is key for non-POEMS, non-HHV8 multicentric cases.
• HHV8-associated cases may involve rituximab, while POEMS subtypes require IL-6 blockade or plasma cell-directed therapy.