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Diagnosis and management of Castleman disease

Jacob D Soumerai1, Aliyah R Sohani, Jeremy S Abramson

  • 1Center for Lymphoma, Massachusetts General Hospital Cancer Center, Boston, MA 02114, USA. jabramson@mgh.harvard.edu.

Cancer Control : Journal of the Moffitt Cancer Center
|October 14, 2014
PubMed

Insights

Castleman disease, a rare lymphoproliferative disorder, presents as localized UCD or systemic MCD. While UCD is treated with surgery, MCD requires evolving systemic therapies like rituximab and targeted agents, with ongoing research needed.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Castleman disease is a rare lymphoproliferative disorder with two main forms: unicentric (UCD) and multicentric (MCD).
  • UCD is localized with a good prognosis, whereas MCD is systemic, often linked to HIV and human herpesvirus 8, carrying significant morbidity and mortality.
  • The management of MCD is complex and evolving, particularly in the context of associated infections and malignancies.

Purpose of the Study:

  • To review the literature on Castleman disease, focusing on clinical presentation, pathological diagnosis, and management strategies.
  • To discuss the distinct characteristics and treatment approaches for unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).
  • To highlight current and emerging therapies for MCD, including targeted agents and supportive care for associated conditions.

Main Methods:

  • A comprehensive review of existing medical literature concerning UCD and MCD.
  • Analysis of clinical presentations and pathological diagnostic criteria for Castleman disease.
  • Examination of associated disorders, including malignancies and autoimmune complications.

Main Results:

  • Surgical resection is the standard treatment for UCD.
  • Systemic therapies, including rituximab, chemotherapy, and antiviral strategies, are essential for MCD management.
  • Novel therapies targeting interleukin-6 show promise for future MCD treatment, alongside careful management of co-existing conditions.

Conclusions:

  • Unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD) are distinct clinicopathologic entities.
  • UCD is generally managed effectively with local therapy, but MCD presents ongoing therapeutic challenges.
  • Recent advancements like rituximab and siltuximab have improved outcomes for MCD, yet further research into its management is crucial.
Abstract

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