Castleman Disease: An overview of symptoms, diagnoses and treatment

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Castleman Disease
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Castleman Disease

• Castleman Disease is a rare disease in which the
  body's disease-fighting network (lymphatic
  system) contains more than required cells
  (overgrowth of cells). It is also known as giant
  lymph node hyperplasia and angiofollicular lymph
  node hyperplasia. Castleman disease can occur in
  a unicentric (localized) or multicentric
  (widespread) form. Multicentric Castleman
  disease can be life-threatening because sometimes
  it is linked with HIV infection. It is also
  associated with other cell-proliferation
  disorders, including cancer of the lymphatic
  system (lymphoma), Kaposi's sarcoma and POEMS
  syndrome.

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Symptoms

• Most patients suffering from unicentric
  Castleman disease don't notice any signs or
  symptoms. The affected lymph node is usually
  located in the chest, neck or abdomen. Signs and
  symptoms 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
• Unexpected weight loss
• Less commonly, fever, night sweats and weakness

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Symptoms
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• Symptoms of multicentric Castleman disease 
• Fever
• Night sweats
• Fatigue and weakness
• Loss of appetite
• Unexpected weight loss
• Enlarged lymph nodes, usually around the neck,
  collarbone, underarm and groin areas
• Enlarged liver or spleen
• Skin rash
• Nerve damage in the hands and feet that leads to
  numbness

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Diagnosis of Castleman disease

• If your doctor suspects unicentric or
  multicentric Castleman disease, he is likely to
  do a physical examination of your lymph nodes, to
  determine their size and consistency. Your doctor
  may then recommend
• Blood and urine tests This is to help rule out
  the possibility of other infections or diseases.
  These tests can also reveal anemia and abnormaliti
  es in blood proteins.
• Imaging tests They are performed to detect
  enlarged lymph nodes, liver or spleen. Along with
  these, CT scan or MRI of your neck, chest,
  abdomen and pelvis may be used.
• Lymph node biopsy It is done to differentiate
  Castleman disease from other types of lymphatic
  tissue disorders, such as lymphoma. A tissue
  sample from an enlarged lymph node is removed and
  taken to a lab for testing.

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Treatments

• Unicentric Castleman disease It can be cured by
  surgically removing the diseased lymph node. A
  major operation may be needed if the lymph node
  is in your chest or abdomen. If surgical removal
  isn't possible, medicines may be used to shrink
  the lymph node following a radiation
  therapy. Multicentric Castleman
  disease Normally, surgery is not the
  right treatment for multicentric Castleman
  disease because of the number of lymph nodes
  involved. However, surgery may be required to
  remove an enlarged spleen. Treatment generally
  involves medications and other therapies to
  manage cell overgrowth including
• Monoclonal antibodies, to block the action of the
  IL-6 protein that contributes to cell overgrowth.
• Chemotherapy, to slow overgrowth of lymphatic
  cells.
• Corticosteroids, to control swelling.
• Antiviral drugs, to block the activity of HHV-8
  or HIV if you have one or both of those viruses.
• Thalidomide, to block the action of the IL-6
  protein.

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Treatments
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• A diagnosis of Castleman disease can be
  demanding because the condition is uncommon and
  often occurs with other serious illnesses, such
  as HIV/AIDS and Kaposi's sarcoma.

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