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This change was made on Mar 28, 2018. See latest version.
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DI 23022.625: Multicentric Castleman Disease

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  • Effective Dates: 01/13/2017 - Present
  • Effective Dates: 03/28/2018 - Present
  • TN 6 (12-11)
  • TN 16 (03-18)
  • DI 23022.625 Multicentric Castleman Disease
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  • MULTICENTRIC CASTLEMAN DISEASE
  • ALTERNATE NAMES
  • MCD; Multicentric Plasma Cell Variant of Castleman’s Disease; Idiopathic Multicentric Castleman’s Disease; HIV- Associated Multicentric Castleman Disease
  • DESCRIPTION
  • Multicentric Castleman Disease (MCD) is a rare disease that affects lymph nodes and other immune cell structures in the body and can severely weaken the immune system. It involves an overproduction of lymphatic cells in the lymph nodes and lymphatic tissues. MCD is associated with HIV infection, infection with the human herpes virus 8 (HHV8), and the development of Kaposi sarcoma. The exact cause of this disease is unknown. Symptoms and signs of MCD include fever, night sweats, loss of appetite, nausea/vomiting, weight loss, cough, shortness of breath, weakness or fatigue from anemia, enlarged lymph nodes (around the neck, collarbone, underarm, and groin area), enlarged spleen and peripheral neuropathy (nerve damage) in the hands and feet leading to numbness and weakness. The multicentric form of Castleman’s disease usually occurs in males between the ages 30 and 50.
  • DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
  • Definitive diagnosis of MCD is made by biopsy or surgical resection of affected lymph nodes. In addition to nodal biopsies, CT scans, MRI, chest x-ray and/or gallium scan may aid in diagnosis. MCD must be differentiated from unicentric Castleman disease, which does not have the same associated morbidity and mortality.
  • ICD-9: 229.0
  • ONSET AND PROGRESSION
  • MCD has a poor prognosis. Death usually results from serious infection, progressive disease, multiple organ failure, or related cancers (i.e. lymphoma or Kaposi sarcoma).
  • TREATMENT
  • Currently, there is no cure for MCD. Treatment is palliative to relieve signs and symptoms of the disease. Corticosteroids are used to control inflammation. Chemotherapy and Rituximab have also been used to treat MCD.
  • Suggested MER for Evaluation:
  • * Documentation of HIV infection
  • * Lymph node biopsy with characteristic histopathology of affected lymph tissue provides the definitive diagnosis of MCD.
  • Suggested Listings for Evaluation:
  • DETERMINATION
  •  LISTING 
  • REMARKS
  • Meets Listing
  •   14.11A
  • 114.11A
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  • Medical Equals
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  • * Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.
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