Castleman Disease | Figure 1

danielcesar

Castleman Disease

Castleman disease (CD) is a rare disease of lymph nodes and related tissues. It is a heterogeneous group of lymphoproliferative disorders that are sometimes associated with human herpesvirus 8 (HHV-8) infection in immunocompromised individuals, such as those with human immunodeficiency virus (HIV) infection.

Treatment varies depending on the type of Castleman disease: unicentric or multicentric. Surgical removal of the involved node is usually curative in unicentric disease, but is rarely curative in multicentric Castleman disease (MCD).The interleukin-6 inhibitor siltuximab is approved for MCD in patients who are negative for HIV and human herpesvirus–8.

The exact cause of Castleman disease is unknown. An increased production of IL-6 by lymph nodes appears to have a role in the development of Castleman disease.

References:

  1. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer. 1956 Jul-Aug. 9(4):822-30.
  2. Yu L, Tu M, Cortes J, Xu-Monette ZY, Miranda RN, Zhang J, et al. Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. Blood. 2017 Mar 23. 129 (12):1658-1668.

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