Unicentric Castlemans disease. Symptoms, diagnostics and therapy.

Area of Science:

• Hematology
• Oncology
• Rare Diseases

Background:

• Castleman disease (CD) encompasses rare, heterogeneous disorders characterized by lymph node abnormalities.
• CD presents as unicentric CD (UCD) affecting a single lymph node station or multicentric CD (MCD) with widespread lymphadenopathy and systemic inflammation.
• Recent advancements include the first diagnostic and treatment guidelines for UCD, published in 2020.

Purpose of the Study:

• To summarize symptoms, diagnostic recommendations, and therapeutic strategies for Castleman disease.
• To highlight the 2020 International, evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease.
• To discuss diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.

Main Methods:

• Review of existing evidence and published international consensus guidelines.
• Analysis of diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
• Examination of therapeutic recommendations for unicentric Castleman disease.

Main Results:

• Complete surgical resection is the preferred first-line therapy for resectable UCD.
• Management of unresectable UCD involves observation for asymptomatic cases or targeted therapies for symptomatic cases.
• Siltuximab is recommended for unresectable UCD with inflammatory syndrome; rituximab, steroids, radiotherapy, or embolization may treat symptomatic unresectable UCD.

Conclusions:

• The 2020 guidelines provide a framework for managing UCD, emphasizing surgical resection when feasible.
• Multicentric CD diagnosis relies on specific criteria, with treatment tailored to disease presentation and severity.
• Effective management strategies for both UCD and MCD are evolving, offering improved patient outcomes.