Historical and pathological overview of Castleman disease

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Castleman disease encompasses diverse lymphoproliferative subtypes with shared lymph node histology but varied clinical presentations and etiologies.
• Unicentric Castleman disease (UCD) is localized, while multicentric Castleman disease (MCD) presents with systemic symptoms and multiple lesions.
• MCD classification includes KSHV/HHV8-related, POEMS syndrome-associated, and idiopathic subtypes, each with distinct characteristics.

Purpose of the Study:

• To elucidate the historical development and evolving understanding of Castleman disease.
• To review current classifications and definitions within the multicentric Castleman disease (MCD) spectrum.
• To summarize key histopathological findings across Castleman disease subtypes.

Main Methods:

• Review of historical literature and seminal reports on Castleman disease.
• Analysis of current diagnostic criteria and classifications for MCD subtypes.
• Synthesis of histopathological features associated with UCD and various MCD forms.

Main Results:

• Castleman disease has evolved from UCD to include complex MCD subtypes.
• MCD is subcategorized based on KSHV/HHV8 status, association with POEMS syndrome, or idiopathic origins.
• Histopathological examination remains crucial for differentiating subtypes and understanding disease mechanisms.

Conclusions:

• A comprehensive understanding of Castleman disease requires appreciating its historical context and diverse subtypes.
• Accurate classification of MCD, considering viral status and associated syndromes, is essential for patient management.
• Further research into idiopathic MCD is needed due to its heterogeneous nature and overlapping features.