Dr Rahul Bhargava Haematologist

Dr. Rahul Bhargava

MBBS, MD, DM (Clinical Haematology)

Principal Director & Chief Hematology at Fortis Hospital

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Dr. Rahul Bhargava

MBBS, MD, DM (Clinical Haematology)

Principal Director & Chief Hematology at Fortis Hospital

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Castleman Disease

Castleman Disease

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Frequently Asked Questions (FAQ) :

No, Castleman Disease is not a type of cancer. However, it can mimic cancer in the way it causes abnormal cell growth in the lymphatic system

Unicentric Castleman Disease can often be cured with surgical removal of the affected lymph node. Multicentric Castleman Disease is more complex and requires ongoing treatment to manage symptoms.

If left untreated, Castleman Disease can lead to serious complications, including organ damage, infections, and in the case of Multicentric Castleman Disease, it can be life-threatening.

While both conditions involve the lymphatic system, Castleman Disease is not a cancer like lymphoma. Lymphoma involves malignant cancerous cells, whereas Castleman Disease involves benign overgrowth of lymphatic cells.

India offers world-class medical care at a fraction of the cost found in Western countries. Specialists like Dr. Rahul Bhargava provide expert care with state-of-the-art technology, making India a top choice for medical tourism.

Understanding Castleman Disease: A Rare but Manageable Condition

Castleman Disease (CD) is a rare and complex disorder that affects the lymph nodes and related tissues. Although it is not classified as a cancer, Castleman Disease can mimic cancer in terms of how it manifests and progresses, leading to abnormal cell growth in the lymphatic system. Understanding the nuances of this disease is critical for both patients and caregivers. Dr. Rahul Bhargava, a leading hematologist and expert in rare blood disorders, offers specialized care for Castleman Disease in India, providing hope and effective treatment options for patients worldwide.

Types of Castleman Disease

Castleman Disease is categorized into two main types, each with distinct characteristics, symptoms, and treatment approaches:

    • Unicentric Castleman Disease (UCD):
      • Characteristics: UCD affects only a single lymph node or a single group of lymph nodes. This type is more common and generally less severe than its multicentric counterpart.
      • Symptoms: Patients may experience localized symptoms such as swelling or a mass in the chest or abdomen, which can cause discomfort, pain, or pressure on surrounding organs.
      • Prognosis: UCD is usually curable, particularly when the affected lymph node is surgically removed.
    • Multicentric Castleman Disease (MCD):
      • Characteristics: MCD affects multiple lymph nodes throughout the body and can lead to systemic symptoms that impact overall health. MCD is further divided into two subtypes:
        • HHV-8-associated MCD: Common in immunocompromised individuals, particularly those with HIV/AIDS.
        • HHV-8-negative/idiopathic MCD: Occurs without the presence of HHV-8 and is less understood.
      • Symptoms: Patients with MCD often experience more severe and widespread symptoms, including fever, night sweats, significant fatigue, anemia, enlarged liver or spleen, and nerve damage.
      • Prognosis: MCD is more challenging to treat and requires ongoing management to control symptoms and prevent complications.
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Causes of Castleman Disease?

The exact cause of Castleman Disease remains largely unknown, making it a challenging condition to study and treat. However, ongoing research has identified some contributing factors:

  • Immune System Dysfunction: One of the primary hypotheses is that Castleman Disease may be related to an overactive immune system, which leads to the uncontrolled growth of cells within the lymph nodes.
  • Viral Infections: In particular, Human Herpesvirus 8 (HHV-8) has been strongly associated with Multicentric Castleman Disease (MCD), especially in patients who are HIV-positive. This viral link is less clear in Unicentric Castleman Disease (UCD).
  • Genetic Factors: While not yet fully understood, there may be genetic predispositions that contribute to the development of Castleman Disease.
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Symptoms of Castleman Disease

    • Unicentric Castleman Disease (UCD):
      • Localized Swelling: A noticeable lump or mass, often in the chest, neck, or abdomen.
      • Pressure Symptoms: Discomfort, pain, or pressure on nearby organs due to the enlarged lymph node.
      • Systemic Symptoms: In some cases, mild fever, fatigue, and weight loss may occur.
    • Multicentric Castleman Disease (MCD):
      • Generalized Lymph Node Enlargement: Swollen lymph nodes in multiple areas, including the neck, armpits, and groin.
      • Systemic Inflammation: Persistent fever, drenching night sweats, and a general feeling of being unwell.
      • Fatigue: Severe tiredness that does not improve with rest.
      • Anemia: A decrease in red blood cells, leading to weakness and pallor.
      • Organ Involvement: Enlargement of the liver or spleen, and in some cases, nerve damage causing pain, numbness, or weakness.
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How is Castleman Disease Diagnosed?

Diagnosing Castleman Disease can be complex due to its rarity and the non-specific nature of its symptoms. A thorough diagnostic process is essential to differentiate Castleman Disease from other conditions with similar presentations, such as lymphoma or autoimmune disorders. The following steps are typically involved:

    • Comprehensive Medical History and Physical Examination:
      A detailed review of the patient’s medical history, symptoms, and a physical examination to assess lymph node enlargement and other related signs.
    • Imaging Studies:
      • CT Scan: Used to identify the size, location, and extent of affected lymph nodes.
      • MRI: Provides detailed images of soft tissues, including lymph nodes and surrounding organs.
      • PET Scan: Helps to determine the activity of the disease and differentiate between benign and malignant conditions.
    • Lymph Node Biopsy:
      The definitive diagnosis of Castleman Disease requires a biopsy of the affected lymph node. This involves removing a small sample of tissue for microscopic examination, which helps to confirm the diagnosis and identify the specific type of Castleman Disease.
    • Laboratory Tests:
      • Blood Tests: To evaluate organ function, check for anemia, and identify markers of inflammation or infection.
      • Viral Tests: Particularly for HHV-8, especially in patients suspected of having Multicentric Castleman Disease.
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Advanced Treatment Options for Castleman Disease

Treatment for Castleman Disease is highly individualized, depending on whether the patient has Unicentric or Multicentric Castleman Disease, as well as the severity of their symptoms.

  • Treatment for Unicentric Castleman Disease (UCD):
    • Surgical Excision: The most common and effective treatment for UCD is the surgical removal of the affected lymph node. In many cases, this leads to a complete cure, with no further treatment required.
    • Radiation Therapy: If surgery is not feasible due to the location of the lymph node, radiation therapy may be employed to shrink the lymph node and alleviate symptoms.
  • Treatment for Multicentric Castleman Disease (MCD):
    • Immunotherapy: The use of monoclonal antibodies like siltuximab (Sylvant) or tocilizumab can help control the overactive immune response that characterizes MCD. These drugs target specific proteins involved in inflammation and lymph node growth.
    • Antiviral Therapy: For patients with HHV-8-associated MCD, antiviral medications may help control the viral load and reduce disease activity.
    • Chemotherapy: In cases where MCD is aggressive or not responsive to other treatments, chemotherapy may be necessary to reduce lymph node size and control symptoms.
    • Corticosteroids: Steroids like prednisone can be used to reduce inflammation and suppress the immune system, providing symptom relief.
    • Supportive Care: Due to the systemic nature of MCD, supportive care is often required to manage symptoms such as anemia, fatigue, and organ involvement. This may include blood transfusions, pain management, and nutritional support.
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Cost of Treatment and Stay in India

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