Castleman Disease

1 What is Castleman Disease?

Castleman disease, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia, is a rare disorder characterized by overgrowth of lymph nodes and associated tissues. The abnormal growth is found in only one part of the body in unicentric form of Castleman disease, while it is more spread out in multicentric form. 

Symptoms and treatment vary with the type of disease. Most of the symptoms are not specific and make diagnosis hard. The underlying cause of the disease is not known. It is found to be associated with other disorders like POEMS syndrome, Kaposi’s sarcoma, and lymphoma. Immunotherapy, chemotherapy, and medications are the common treatment methods followed. Unicentric form can be successfully treated by surgery.

2 Symptoms

Symptoms of Castleman disease may vary depending on the type of the disease.

Castleman disease is categorized into two based on regions affected by growth of the lymph nodes. 

Unicentric – overgrowth of lymph nodes is found only in one part of the body. 

Multicentric – as the name indicates, it affects more than one lymph node and tissues. There are three different types of multicentric: 

Castleman disease –Multicentric Castleman disease without POEMS syndrome

  • with POEMS syndrome that involves areas of abnormal bone 
  • with POEMS syndrome but without involvement of abnormal bone lesions. 
  • Unicentric Castleman disease is often found in chest, neck, or abdomen. Characteristic symptoms of unicentric form are

Discomfort or pressure in the chest

  • Difficulty in breathing if the enlarged lymph nodes are in chest
  • Difficulty in eating and feeling of fullness if the enlarged nodes are in abdomen
  • Enlarged lump in armpit, neck, or groin
  • Sudden weight loss 
  • Fever and night sweats are rare in unicentric form of the disease

Common symptoms of multicentric form of Castleman disease include 

  • Fever
  • Night sweats
  • Lack of appetite
  • Enlarged lymph nodes around neck, collar bone, underarm and groin
  • Enlargement of liver and spleen

Some people may have numbness in limbs due to nerve damage. Skin rashes are also seen as a rare symptom. 

3 Causes

The actual cause of Castleman disease is not known. In many cases, this disease is found to be associated with human herpes virus 8 (HHV-8) infection. In this case, immune deficiency or infection allows the virus to escape detection by the immune system. 

The virus replicates in the lymph nodes and triggers excess production of inflammatory chemicals. These chemicals cause the abnormal overgrowth of lymph nodes. In majority of cases of Castleman disease the cause for release of inflammatory chemicals is not known. 

Three different processes are implied in the cause of overgrowth of lymph nodes. 

  • Autoimmune mechanism
  • Inflammatory chemical secretion by benign tumors
  • Viral signaling

4 Making a Diagnosis

The unicentric form of Castleman disease remains asymptomatic in many cases, and is often diagnosed during screening for other concerns. Physical examination of the lymph nodes to assess the size and consistency of the growth is the first step in diagnosis. 

Blood tests and urine tests help to rule out chances of other conditions that lead to similar symptoms. Enlargement of lymph nodes, liver, and spleen is noted with the help of imaging techniques. CT scan, MRI, and PET scan help in visualizing the abnormalities of these organs. 

Biopsy of the affected lymph tissue helps to differentiate Castleman disease from lymphomas.

The unicentric form of Castleman disease remains asymptomatic in many cases, and is often diagnosed during screening for other concerns.

5 Treatment

Treatment of Castleman disease depends on the type of disease:

  • Unicentric Castleman disease – surgical removal of the affected lymph node is the most common treatment method for unicentric form. Medications are prescribed if surgery is not feasible. Medication helps to shrink the size of the growth. Radiation therapy is also used to clear the defective lymph node. 
  • Multicentric Castleman disease – as a large number of lymph nodes are involved in this condition, surgery is not an option. Surgery is suggested to remove enlarged spleen. Medications are prescribed to control the growth the node. Treatment also depends on the presence of viral infections and extent of disease. 

Monoclonal antibodies are used to block the action of inflammatory chemicals that result in overgrowth of lymph nodes. Siltuximab is a common monoclonal antibody used as initial treatment, particularly if there is no viral infection. 

Chemotherapy is recommended when the patient does not respond to monoclonal antibodies. Corticosteroids are used to reduce inflammation, while antiviral drugs controls viral infection. Thalidomide is another medication used to block the action of inflammatory chemicals. 

6 Prevention

There are no standard preventive measures for Castleman disease as the actual cause of the disease is not known. Take adequate measures to control action of inflammatory chemicals to prevent the condition in the presence of viral infections.

7 Alternative and Homeopathic Remedies

There is very little information regarding alternative remedies for controlling Castleman disease. Anti-inflammatory diet is suggested to alleviate the symptoms of this condition.

8 Lifestyle and Coping

Discussing the experiences and feeling with a doctor or a support group help to cope with Castleman disease better.

Castleman disease is often associated with other serious conditions and infections like Kaposi’s sarcoma, and HIV infection. This is challenging for patients and their family. Discussing the experiences and feeling with a doctor or a support group help to cope with the condition better.

9 Risks and Complications

Castleman disease increases the risk of lymphomas. It may also lead to potentially life-threatening conditions like multiple organ damage and cancer. Moreover, it is associated with HIV and herpes infections.

10 Related Clinical Trials