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Hematology/Oncology

Castleman Disease

Castleman disease is a lymphoproliferative disorder with a nondefinite etiology and can be classified into subtypes based on clinical and histological manifestations. Therefore, this condition's high morbidity and complications can be avoided with early diagnosis and prompt treatment. This activity explains the evaluation and treatment of Castleman disease and highlights the role of the interprofessional team in evaluating and treating patients with this condition. Objectives: Describe the patho

v1 · Reviewed Nov 7, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • In extreme cases of iMCD, multiple organ failure with renal insufficiency can ensue, often resulting in death.
  • Therefore, this condition's high morbidity and complications can be avoided with early diagnosis and prompt treatment.
  • The diagnosis of Castleman disease is always a challenge in clinical experience, as it does not have specific features that could be distinguished from other diseases causing lymphadenopathies.
  • Histopathologic screening of lymph nodes is done to assess a single node involvement (suggestive of UCD) or a multimode involvement (suggesting MCD) after excluding other infectious, malignant, and autoimmune disorders that exhibit their characteristic features in the lymph nodes.
  • The lymph node size must be enlarged.
  • Minor Criteria Laboratory Elevated CRP or ESR Anemia Thrombocytopenia or thrombocytosis Hypoalbuminemia Renal dysfunction or proteinuria Polyclonal hypergammaglobulinemia Clinical B symptoms: fever, weight loss, night sweats, fatigue Splenomegaly or Hepatomegaly Fluid accumulation( edema, anasarca, pleural effusion)
  • IL-6 has proven to be a specific biomarker for this disease, contributing to its pathogenesis, symptomatology, and histopathology.
  • The patient must also undergo serology tests for HIV and HHV-8 and search for HHV-8 DNA in peripheral blood by polymerase chain reactive (PCR) to discover associations with CD.
  • An evaluation including serum protein electrophoresis, bone marrow examination, CT scan of the chest, abdomen, and pelvis, radiographic skeletal survey, and gallium scan provides essential information in detecting the extent of the disease and also distinguishes multicentric from localized Castleman disease.
  • Definitive diagnosis should only be made when all other causes of lymphadenopathy are investigated and excluded.
Diagnostic Criteria
patient meets both major criteria, at least two of the minor criteria and one laboratory abnormality.
Major Criteria
Histopathologic screening of lymph nodes is done to assess a single node involvement (suggestive of UCD) or a multimode involvement (suggesting MCD) after excluding other infectious, malignant, and autoimmune disorders that exhibit their characteristic features in the lymph nodes.
  • As a unicentric Castleman disease lesion is localized, complete surgical resection of the tumor is the best treatment available.
  • Monoclonal antibodies interrupting the IL-6 signaling cascade, including the anti–IL-6R antibody Tocilizumab and Siltuximab, an anti–IL-6 antibody, tend to be highly effective and are considered to be the first-line therapy, especially for highly symptomatic patients without being HIV or HHV-8 positive.
Tocilizumab
Not specified in source
Adult
Not specified in source
Renal
Not specified in source
Duration
Not specified in source
Siltuximab
Not specified in source
Adult
Not specified in source
Renal
Not specified in source
Duration
Not specified in source
  • Antiviral therapy comprising of ganciclovir, foscarnet, and cidofovir is proven effective against HHV-8 replication pathways.
  • Novel immunomodulatory agents like rituximab, thalidomide, bortezomib, IL-1 antagonist anakinra, and interferon-alpha have somehow shown promising results as well with minimum side effects.
  • Chemotherapy and immunomodulatory drugs are best reserved for the relapse scenarios.
  • Around 30% of patients with POEMS disease also have multicentric CD, more precisely the HHV-8 positive variant.
  • Patients with MCD should go for regular checkups to avoid any fatal complications.
  • paraneoplastic pemphigus (PNP) and lung abnormalities like bronchiolitis obliterans (BO) are the two most critical rare diseases occurring with the UCD.
  • In contrast, hemopoietic (thromboembolic disorders and inflammatory reactions) and internal organ involvement (renal, hepatic) are mainly associated with MCD.
  • The survival rate is significant in patients without these complications.
  • Therefore, early diagnosis for these manifestations is necessary for tailoring appropriate therapy options, leading to a better prognosis.
  • Both UCD and MCD can sometimes progress to non-Hodgkin lymphoma (NHL).
  • review
    Castleman Disease. StatPearls [Internet] — Nimra Ehsan, Farah Zahra — StatPearls Publishing, 2023

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov