D47.Z2 Castleman disease

ICD10CM code

#### Name of the Condition
- Castleman disease
- Also known as Castleman syndrome, giant lymph node hyperplasia, angiofollicular lymph node hyperplasia.

#### Summary
Castleman disease is a rare disorder characterized by non-cancerous growths that can develop in the lymph node tissue. It can occur in a localized (unicentric) form or can be systemic (multicentric), affecting multiple regions of the body.

#### Causes
The exact cause of Castleman disease is not fully understood. However, it has been associated with human herpesvirus 8 (HHV-8) and may have an underlying immune system dysfunction.

#### Risk Factors
- Infection with human herpesvirus 8 (HHV-8)
- HIV/AIDS infection, particularly for multicentric Castleman disease
- Immunocompromised individuals

#### Symptoms
- Unicentric: Swollen lymph nodes, usually asymptomatic; sometimes fever, fatigue, or weight loss.
- Multicentric: Fever, night sweats, unexplained weight loss, enlarged liver or spleen, nerve damage, and weakened immune function.

#### Diagnosis
- Physical examination and medical history
- Blood and urine tests
- Imaging studies like CT or MRI scans
- Lymph node biopsy to examine tissue

#### Treatment Options
- **Unicentric:** Surgical removal of the affected lymph node is often curative.
- **Multicentric:** May involve immunotherapy, antiviral drugs, corticosteroids, chemotherapy, or monoclonal antibodies to manage symptoms and control the disease.

#### Prognosis and Follow-Up
- Prognosis varies based on the form; generally excellent for unicentric with surgical treatment.
- Multicentric form can be chronic and requires ongoing management.
- Regular follow-ups are essential to monitor disease progression, particularly in multicentric cases.

#### Complications
- Multicentric Castleman disease can lead to severe infections due to immune system impairment.
- Increased risk of developing lymphoma.
- Organ dysfunction if left untreated.

#### Lifestyle & Prevention
- There are no known preventive measures due to unknown cause.
- Lifestyle changes are not specifically recommended, but maintaining a healthy immune system can be beneficial.

#### When to Seek Professional Help
- Persistent swelling of lymph nodes
- Symptoms such as prolonged fever, night sweats, or unexplained weight loss

#### Additional Resources
- Castleman Disease Collaborative Network (CDCN): [Website](https://www.cdcn.org/)
- American Cancer Society: Information on lymphatic diseases

#### Tips for Medical Coders
- Ensure correct code assignment between unicentric and multicentric forms, as they may have different implications for treatment and prognosis.
- Avoid errors by checking for specific diagnosis and documented symptoms; D47.Z2 is used for unspecified types.

Medical Policies and Guidelines for Castleman disease

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