Castleman disease, a dilemma in HIV patients: A clinical presentation and diagnostic approach

Abstract

The first case of CD was described in 1950. Castleman disease (CD) is a rare group of heterogeneous lymphoproliferative disorders that share histopathological features related to the increased release of cytokines, particularly interleukin 6 (IL-6). CD has many etiologies, presentations, treatments, and outcomes. CD can be classified as unicentric Castleman disease (UCD) or Multicentric Castleman disease (MCD). UCD affects a single group of lymph nodes, whereas MCD affects multiple groups of lymph nodes. There are no known risk factors for UCD. MCD can either be idiopathic or associated with an immunocompromised state which may lead to the proliferation of B-lymphocytes as well as plasma cells in lymphoid organs in different body regions. Moreover, MCD has a preference for male over female patients. The average age for diagnosing MCD is the sixth decade. Contrarily, UCD has no gender preference and it is typically diagnosed in the fourth decade. UCD can present as asymptomatic lymphadenopathy or as a progressive enlargement of the lymph nodes. UCD is incidentally diagnosed on imaging of the chest or abdomen. MCD may present with lymphadenopathy, cytopenia or constitutional symptoms. The treatment options for UCD and MCD may include monoclonal antibodies, cytotoxic therapy, chemotherapy, radiotherapy or surgery.