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Unicentric Castlemans disease. Symptoms, diagnostics and therapy

Zdeněk Adam, Zdeněk Řehák, Zuzana Adamová

    Vnitrni Lekarstvi
    |April 23, 2022
    PubMed

    Insights

    Castleman disease (CD) is a rare disorder affecting lymph nodes. New guidelines offer diagnostic and treatment strategies for unicentric CD (UCD) and multicentric CD, focusing on surgical resection and targeted therapies.

    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.

    Keywords:
    Castleman diseaseCastleman’s diseaserituximabsiltuximab

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