GeneHealth - Your precision medicine

Castleman Disease

Disease Details

Family Health Simplified

Buy Membership

Description: Castleman disease is a rare disorder characterized by non-cancerous growths that can develop in the lymph node tissue and can affect a single lymph node or multiple lymph nodes throughout the body.
Type: Castleman disease is not typically inherited and usually does not follow a genetic transmission pattern. It is generally classified into two main types: unicentric Castleman disease (UCD), which affects a single lymph node or a single region of lymph nodes, and multicentric Castleman disease (MCD), which affects multiple lymph node regions and can have more severe systemic symptoms.
Signs And Symptoms: Castleman disease, also known as Castleman syndrome, can present with a range of signs and symptoms depending on whether it is unicentric or multicentric.

### Unicentric Castleman Disease (UCD)
- **Localized Lymph Node Enlargement**: Usually in the chest, abdomen, or neck.
- **Symptoms Due to Mass Effect**: Can cause breathing difficulties, swallowing issues, or compression of nearby organs.
- **Systemic Symptoms**: Generally absent, but some patients may experience mild systemic symptoms.

### Multicentric Castleman Disease (MCD)
- **Generalized Lymph Node Enlargement**: Enlarged lymph nodes in multiple regions.
- **Systemic Symptoms**:
- **Fever**
- **Night Sweats**
- **Weight Loss**
- **Fatigue**
- **Hematological Abnormalities**:
- **Anemia**
- **Elevated inflammatory markers**
- **Organomegaly**: Enlargement of liver or spleen.
- **Skin Rashes**: In some cases.

Patients with MCD may also have associated conditions such as Kaposi's sarcoma or herpesvirus infection (HHV-8 associated MCD).

Timely diagnosis and treatment are important, especially for MCD, due to its systemic nature and potential for serious complications.
Prognosis: Castleman disease prognosis can vary significantly depending on the type and severity of the condition. There are two main forms: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).

- **Unicentric Castleman Disease (UCD):** The prognosis for UCD is generally favorable. Most patients can be cured with surgical removal of the affected lymph node. Recurrence is rare, and long-term outcomes are typically good.

- **Multicentric Castleman Disease (MCD):** The prognosis for MCD is more variable and can be challenging. It often depends on the underlying causes, such as HIV or HHV-8 infection, and the presence of other associated conditions. Treatment can include medications like antivirals, immunotherapy, and chemotherapy. Some patients may achieve remission, but MCD can be chronic and relapsing, leading to a more guarded prognosis.

Overall, early diagnosis and treatment are crucial in managing Castleman disease effectively.
Onset: Castleman disease can have a variable onset. It may occur at any age, although the typical onset is in young adults. The disease can present with flu-like symptoms such as fever, fatigue, and weight loss, or with enlarged lymph nodes. The onset can be sudden or gradual, depending on the subtype (unicentric or multicentric).
Prevalence: The prevalence of Castleman disease is not precisely known due to its rarity and the variability of its presentation. However, it is generally considered to be a rare condition, with an estimated incidence of about 21 to 25 cases per million people worldwide.
Epidemiology: Castleman disease is a rare disorder characterized by non-cancerous growths that can develop in the lymph node tissue. There are two main types: Unicentric Castleman Disease (UCD), which affects a single lymph node, and Multicentric Castleman Disease (MCD), which affects multiple lymph nodes and can be associated with more severe symptoms.

Epidemiology:
1. **Incidence**: The exact incidence is not well-documented due to its rarity, but it is estimated to affect 21 to 25 per million people worldwide.
2. **Age and Gender**: UCD tends to affect young adults and can occur in both men and women equally. MCD typically affects older adults and may have a slightly higher prevalence in males.
3. **Geographic Distribution**: There is no clear geographic or ethnic predisposition, although certain subtypes of MCD are more common in specific populations, such as those infected with HIV or HHV-8 (Human Herpesvirus 8).
4. **Risk Factors**: Known risk factors include HIV infection and immunosuppression, particularly for HHV-8-associated MCD.

The term "nan" in your query is unclear in this context. If it refers to "not a number" or a data-related concept, please clarify your intent so I can provide more relevant information.
Intractability: Castleman disease (CD) is not typically classified as intractable, but its management can be complex and challenging, varying with the subtype. There are two main subtypes: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD can often be treated effectively with surgical removal of the affected lymph node. MCD, particularly when associated with HIV or HHV-8 infection, may require systemic treatments like immunotherapy, antiviral therapy, and chemotherapy. While treatment can be challenging and requires careful management, especially for MCD, it is not considered uniformly intractable.
Disease Severity: Castleman disease can vary in severity. It exists in two primary forms: unicentric and multicentric.

- Unicentric Castleman disease involves a single lymph node and is often less severe. It usually responds well to surgical removal of the affected lymph node and has a good prognosis.

- Multicentric Castleman disease affects multiple lymph nodes and can be more severe. It can lead to systemic symptoms and is sometimes associated with other conditions such as HIV or Kaposi sarcoma-associated herpesvirus (KSHV). Treatment may involve medications such as corticosteroids, immunotherapy, and chemotherapy, and the prognosis can be more variable compared to the unicentric form.
Healthcare Professionals: Disease Ontology ID - DOID:0111157
Pathophysiology: Castleman disease is a rare and complex lymphoproliferative disorder characterized by the abnormal growth of lymphatic tissue. The pathophysiology of Castleman disease varies based on its type:

1. **Unicentric Castleman Disease (UCD)**: This form affects a single lymph node or a single group of lymph nodes. The exact mechanism is not entirely understood, but it often involves localized, abnormal immune responses leading to the overproduction of cytokines, such as interleukin-6 (IL-6), which stimulate lymph node enlargement.

2. **Multicentric Castleman Disease (MCD)**: This form affects multiple lymph nodes and can be more severe. MCD often correlates with systemic symptoms and is sometimes associated with human herpesvirus 8 (HHV-8) infection, especially in HIV-positive individuals. The virus can infect lymphatic cells, leading to the overproduction of IL-6 and other inflammatory cytokines, causing widespread lymph node enlargement and systemic inflammation. In HHV-8-negative MCD, the pathogenesis remains unclear but is thought to involve dysregulated immune responses as well.

In both forms, the overproduction of cytokines is central to disease progression, leading to the proliferation of lymphoid tissue and associated clinical manifestations.
Carrier Status: Castleman disease is not a genetic disorder; therefore, there is no carrier status associated with it. It is a rare condition involving an overgrowth of cells in the body's lymph nodes.
Mechanism: Castleman disease (CD) is a rare lymphoproliferative disorder. Its exact mechanism is not fully understood, but it's generally characterized by lymph node enlargement and can involve multiple organs. There are two main types: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).

### Mechanism:
1. **Unicentric Castleman Disease (UCD):**
- **Local Lymph Node Hyperplasia:** This type primarily involves a single lymph node or a single region of lymph nodes. The cause is often unknown, although localized production of cytokines may play a role.

2. **Multicentric Castleman Disease (MCD):**
- **Systemic Inflammation and Cytokines:** MCD affects multiple lymph node regions and often exhibits systemic symptoms due to the overproduction of pro-inflammatory cytokines, particularly interleukin-6 (IL-6). This leads to a systemic inflammatory response.

### Molecular Mechanisms:
1. **Cytokine Dysregulation:**
- IL-6 is a key driver in many cases of MCD, contributing to symptoms such as fever, night sweats, and weight loss. Elevated IL-6 levels can cause B-cell proliferation and systemic inflammation.

2. **Human Herpesvirus 8 (HHV-8):**
- In HHV-8 positive cases of MCD, the virus infects B cells and induces cytokine production, including viral IL-6, which mimics the action of human IL-6, further driving disease pathology.

3. **Genetic and Environmental Factors:**
- Although less understood, genetic predispositions and other environmental triggers may also contribute to the dysregulated immune response in both UCD and MCD.

4. **B-cell Activation and Proliferation:**
- The activation and proliferation of B-cells, influenced by cytokines and viral infections, result in lymph node enlargement and systemic symptoms.

Understanding these mechanisms is crucial for the development of targeted therapies, like anti-IL-6 monoclonal antibodies, which are currently used to treat MCD.
Treatment: Treatment for Castleman disease typically varies depending on whether it's unicentric (localized to a single set of lymph nodes) or multicentric (affecting multiple lymph node regions).

**Unicentric Castleman Disease:**
- **Surgical Removal:** The primary treatment is often surgical removal of the affected lymph node.
- **Radiation Therapy:** May be used if surgery isn't an option.

**Multicentric Castleman Disease:**
- **Medications:**
- **Monoclonal Antibodies:** Drugs like siltuximab or tocilizumab target interleukin-6 (IL-6) to reduce inflammation and disease activity.
- **Immunosuppressants:** Corticosteroids or other immunosuppressive drugs can help control symptoms.
- **Chemotherapy:** Used in more severe cases to manage excessive growth of immune cells.
- **Antiviral Therapy:** If associated with HHV-8 (Human Herpesvirus 8), antiviral drugs may be employed.

Treatment plans should be personalized based on the patient's specific condition and response to previous treatments. Regular monitoring and follow-up care are essential to manage the disease effectively.
Compassionate Use Treatment: Compassionate use treatment for Castleman disease may involve experimental therapies that are not yet widely approved but show promise in treating the condition. Off-label or experimental treatments can include:

1. **Siltuximab (Sylvant)**: Although primarily approved for multicentric Castleman disease (MCD) not associated with HIV or HHV-8, it may be used off-label for other subtypes.

2. **Tocilizumab (Actemra)**: An IL-6 inhibitor approved for rheumatoid arthritis, but sometimes used off-label in Castleman disease.

3. **Rituximab (Rituxan)**: An anti-CD20 monoclonal antibody often used in HIV-associated Castleman disease or as part of combination therapy.

4. **Thalidomide or Lenalidomide**: Immunomodulatory drugs that have shown efficacy in certain patients.

5. **Bortezomib (Velcade)**: A proteasome inhibitor used experimentally for difficult cases.

Experimental treatments for Castleman disease are generally considered on a case-by-case basis, often when standard therapies are ineffective.
Lifestyle Recommendations: Castleman disease is a rare disorder involving an overgrowth of cells in the body's lymph nodes. Lifestyle recommendations for managing Castleman disease primarily focus on supporting overall health and helping to manage symptoms. Here are some general lifestyle recommendations:

1. **Regular Medical Follow-Up**: Stay in regular contact with your healthcare team to monitor the disease's progression and adjust treatment plans as necessary.

2. **Balanced Diet**: Consume a diet rich in fruits, vegetables, whole grains, lean proteins, and healthy fats to support overall health and potentially boost your immune system.

3. **Adequate Hydration**: Drink plenty of water to stay hydrated, which can help with overall bodily functions.

4. **Regular Exercise**: Engage in moderate physical activity as recommended by your doctor to maintain strength and overall well-being.

5. **Stress Management**: Practice stress-reducing techniques such as yoga, meditation, or deep-breathing exercises to help manage mental health.

6. **Sleep Hygiene**: Ensure you get adequate and quality sleep each night to help your body repair and function optimally.

7. **Avoid Infections**: Take precautions to avoid infections, as your immune system may be compromised. This includes practicing good hygiene and avoiding close contact with sick individuals.

8. **Smoking and Alcohol**: Avoid smoking and limit alcohol consumption, as these can have negative effects on your immune system and overall health.

Always consult your healthcare provider for personalized advice tailored to your condition and overall health status.
Medication: Castleman disease treatment varies depending on the type and severity but often involves medications such as:

1. **Monoclonal Antibodies**: Siltuximab and rituximab are commonly used to target specific proteins involved in the disease.
2. **Immunosuppressive Drugs**: Corticosteroids like prednisone are used to reduce inflammation.
3. **Chemotherapy**: Drugs like cyclophosphamide and doxorubicin may be used particularly in multicentric forms or in cases associated with lymphoma.
4. **Antiviral Therapy**: For cases associated with human herpesvirus 8 (HHV-8), antiviral medications might be recommended.
5. **Immunomodulators**: Drugs like thalidomide might be used to modify the immune response.

The choice of medication depends on factors such as whether the disease is unicentric or multicentric, the patient’s overall health, and the presence of any associated conditions.
Repurposable Drugs: Some repurposable drugs for Castleman disease (CD) include:

1. **Tocilizumab**: An IL-6 receptor antagonist originally used for rheumatoid arthritis.
2. **Siltuximab**: An anti-IL-6 monoclonal antibody that has shown efficacy in CD.
3. **Thalidomide**: Initially developed as a sedative, it shows immunomodulatory effects.
4. **Lenalidomide**: A more potent derivative of thalidomide, used for multiple myeloma.

These therapies target inflammatory pathways implicated in CD pathogenesis.
Metabolites: Castleman disease is a rare disorder characterized by non-cancerous growths that can develop in the lymph node tissue. Currently, specific metabolites associated with Castleman disease are not well characterized or widely used in clinical practice. The disease involves abnormal B-cell proliferation and immune system dysregulation, which can lead to elevated levels of certain cytokines, such as interleukin-6 (IL-6). However, detailed metabolic profiling specific to Castleman disease is not a routine part of diagnosis or management.
Nutraceuticals: Nutraceuticals' role in Castleman disease is still under research. There's no conclusive evidence supporting their effectiveness in treating this rare lymphoproliferative disorder. Patients with Castleman disease should follow conventional medical treatments and consult healthcare providers before using any supplements or nutraceuticals.
Peptides: Castleman disease is a rare disorder characterized by non-cancerous growths (tumors) that may develop in the lymph node tissue. There are no peptides specifically associated with Castleman disease. The disease involves overproduction of certain proteins by parts of the immune system, often leading to symptoms like enlarged lymph nodes, fever, and fatigue.

Treatment may involve targeting the excess interleukin-6 (IL-6) produced in the body, which contributes to the disease’s symptoms. Monoclonal antibodies, like siltuximab, which specifically block IL-6, can be used in treatment. Research does explore the role of peptides and proteins in disease mechanisms and treatments, but there are no specific peptides currently central to the standard understanding or management of Castleman disease.