Castleman Disease – What is it, Symptoms and Treatments!

Castleman’s Disease – What it is, Symptoms and  Drug Treatments. Additionally, Castleman Disease is a rare disorder that involves an overgrowth (proliferation) of cells in your body’s disease -fighting network (lymphatic system).

Also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, Castleman disease can occur either localized (unicentric) or diffused (multiccentric). Treatment and outlook vary depending on what type of Castleman Disease you have. The localized type can usually be successfully treated with surgery.

Sometimes associated with HIV infection, multicentric Castleman Disease can be fatal. Multicentric Castleman Disease is also associated with other disorders of cell proliferation, including cancer of the lymphatic system (lymphoma), Kaposi’s sarcoma, and POEMS syndrome.

Causes of Castleman Disease:  It is unclear what causes Castleman Disease . However, infection with a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease. The HHV-8 virus has also been linked to the development of Kaposi’s sarcoma, a cancerous tumor of the blood vessel walls that can be a complication of HIV/AIDS.

Studies have found that HHV-8 is present in HIV-positive people who have Castleman Disease and in 40 to 50 percent of HIV-negative people who have Castleman Disease . The precise role of the HHV-8 is unclear. But it appears that immune system cells are malfunctioning to reproduce quickly. Immune system cells produce a protein called interleukin-6 (IL-6) that contributes to lymph cell overgrowth.

Symptoms  of Castleman Disease:  There are two basic types of Castleman Disease :

  • Unicentric Castleman disease . This localized form of the disease affects only one gland (lymph node) in your lymphatic system.
  • Castleman’s multicultural disease. This type affects multiple lymph nodes and lymph tissues, and can severely weaken your immune system .

Multicultural Castleman disease can be classified as:

  • Multicentric Castleman disease without POEMS syndrome
  • Multicentric Castleman disease with POEMS syndrome involving areas of abnormal bone (osteosclerotic lesions)
  • Multicentric Castleman disease with POEMS syndrome without osteosclerotic lesions

Unicentric Castleman Disease:  Many people with unicentric Castleman Disease do not see any signs or symptoms. The diseased lymph node is usually located in the chest, neck, or abdomen. When signs and symptoms are present, they can include:

  • A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
  • An enlarged lump under the skin in the neck, groin, or armpit
  • involuntary weight loss
  • Less commonly, fever, night sweats, and weakness

Multicentric Castleman Disease:  Most people with multicentric experience of Castleman Disease :

  • Fever
  • night sweat
  • fatigue and weakness
  • loss of appetite
  • involuntary weight loss
  • Enlarged lymph nodes, usually around the neck, collarbone, armpits, and groin areas
  • Enlarged liver or spleen

Other less common symptoms include:

  • Nerve damage in the hands and feet that leads to numbness (peripheral neuropathy)
  • Rash

When to See a Doctor:  If you notice an enlarged lymph node on the side of your neck or in your armpit, collarbone or groin area, talk to your doctor. Also call your doctor if you have a persistent feeling of fullness in your chest or abdomen, fever, fatigue, or unexplained weight loss.

Castleman Disease Risk Factors: Castleman  Disease canaffect anyone. But the average age of people diagnosed with unicentric Castleman Disease is 35. Most people with the multicentric form are in their 50s and 60s. The multicentric form is also slightly more common in men than in women. The only known risk factor for Castleman Disease appears to be HIV/AIDS.

Complications of Castleman Disease:  People with unicentric Castleman Disease usually do well once the affected lymph node is removed. However, having Castleman Disease can increase your risk of lymphoma. Complications of multicultural Castleman disease can be life-threatening and can include:

  • Infection leading to multiple organ failure
  • Cancer, such as lymphoma or Kaposi’s sarcoma

The outlook for people with multicentric Castleman Disease varies, depending on the nature of the disease. The presence of HIV/AIDS tends to worsen the outcome. Research also indicates that people who have multicentric Castleman Disease with POEMS syndrome that do not involve bone lesions may have worse outcomes, whereas people who have multicentric Castleman Disease with the bone lesion variant of POEMS syndrome tend to improve.

Preparing for Your Appointment:  You may be referred to a doctor who specializes in treating blood disorders (haematologist). What can you do:

  • Write down the symptoms you experienced and for how long.
  • Write down key medical information, including other conditions.
  • Make a list of all the medications, vitamins and supplements you are taking.

Questions to ask your doctor:

  • What is the most likely cause of my signs and symptoms?
  • What types of tests do I need? Do they require any special preparation?
  • What treatment do you recommend? Do I need surgery?

In addition to the questions you’ve prepared to ask your doctor, don’t hesitate to ask other questions during your appointment.

What to Expect from Your Doctor: Your doctor  is likely to ask you a series of questions. Being ready to answer them can make time to get past the points you want to spend more time on. You may be asked:

  • Do you have other health conditions such as HIV/AIDS or Kaposi’s sarcoma?
  • When did you first start experiencing symptoms?
  • Were your symptoms continuous or occasional?
  • How severe are your symptoms?
  • Does anything seem to improve your symptoms?
  • What, if anything, seems to make your symptoms worse?

Tests and Diagnosis of Castleman Disease:  People with unicentric Castleman Disease usually do not see any signs or symptoms. The diseased lymph node may be found during screening or treatment for another disease. If unicentric or multicentric Castleman Disease is suspected , your doctor will likely start with a thorough physical exam of your lymph nodes to determine their size and consistency. Your doctor may then recommend:

  • Blood and urine tests, to help rule out other infections or illnesses . These tests can also reveal anemia and blood protein abnormalities that are sometimes characteristic of Castleman Disease .
  • Imaging tests, to detect enlarged lymph nodes, liver or spleen. Computed tomography or MRI of the neck, chest, abdomen, and pelvis may be used. Positron emission tomography (PET) scans can also be used to diagnose Castleman Disease and assess whether a treatment is effective.
  • Lymph node biopsy, to differentiate Castleman disease from other types of lymph tissue disorders, such as lymphoma. A tissue sample from an enlarged lymph node is removed and examined in the laboratory. Depending on the location of the lymph node, the biopsy may be performed under local anesthesia or more extensive surgery.

Castleman Disease  Treatments : Treatment depends on the type of Castleman Disease you have.

Unicentric Castleman Disease : Unicentric Castleman  Disease can be cured surgically by removing the diseased lymph node. If the lymph node is in the chest or abdomen — which is often the case — major surgery may be necessary. If surgical removal is not possible, medication may be used to shrink the lymph node. Radiation therapy can also be an effective way to destroy affected tissue. You will need follow-up tests, including imaging, to check for relapse.

Useful links: 

Multicentric Castleman Disease:  Surgery is usually not an option for multicultural Castleman disease due to the number of lymph nodes involved. However, surgery to remove an enlarged spleen may be an option to help relieve symptoms. Treatment usually involves drugs and other therapies to control the cell’s overgrowth. Specific treatment depends on the extent of your illness and whether you have HIV or HHV-8 infection or both. Options include:

  • Monoclonal antibodies, to block the action of the IL-6 protein that contributes to cell overgrowth. Your doctor may recommend initial treatment with a monoclonal antibody, such as siltuximab (Sylvant), if you don’t have organ damage or HIV or HHV-8 infection.
  • Chemotherapy, to slow the overgrowth of lymph cells. Your doctor may recommend adding chemotherapy if the disease does not respond to monoclonal antibodies or if you have organ failure.
  • Corticosteroids, to control inflammation.
  • Antiviral drugs, to block the activity of HHV-8 or HIV if you have one or both viruses.
  • Thalidomide (Thalomid), to block the action of the IL-6 protein. Thalidomide is an immune system modulator that has been shown to be effective in inducing remission in Castleman Disease .

Similar Posts

Leave a Reply

Your email address will not be published. Required fields are marked *