Giant lymph node hyperplasia

Giant lymph node hyperplasia is a rare disorder of lymphatic system which is also called as Castleman’s disease or angiofollicular lymph node hyperplasia. It can be localized or widespread, but it’s not a cancer. It is a lymphoproliferative disorder characterized by an abnormal or over growth of lymph system is observed. Many people with this condition eventually develop lymphomas (cancer of lymph nodes). Commonly it occurs in the chest, abdomen, neck, axilla, pelvis, and pancreas.

Signs and Symptoms

The signs and symptoms differ based on the type. The symptoms of localized type include:

  • A feeling of fullness
  • Difficulty in breathing
  • Difficulty in eating
  • Enlarged lump (neck, groin or armpit)
  • Unintended weight loss
  • Less commonly, fever, night sweats and weakness

The signs and symptoms of multicentric type include:

  • Fever
  • Loss of appetite
  • Unintended weight loss
  • Night sweats
  • Fatigue and weakness
  • Enlarged lymph nodes (neck, collar bone, underarm and groin areas)
  • Enlarged liver or spleen
  • Less commonly, skin rash, nerve damage that leads to numbness

Causes of Giant Lymph Node Hyperplasia

The causeof giant lymph node hyperplasia is not clear. Herpes virus 8 (HHV-8) is associated with multicentric giant lymph node hyperplasia. The HHV-8 develops Kaposi’s sarcoma (cancerous tumor of blood vessel walls). Interleukin-6 (IL-6) produced by the immune system, contributes to the overgrowth of lymphatic cells.

The diagnostic tests for giant lymph node hyperplasia include

Blood and Urine Tests :Infections and other type of diseases can be identified by these tests. These tests will be useful to identify abnormalities in blood proteins.
Imaging : These are used to detect the enlarged lymph nodes. CT and MRI are used for imaging studies of the lymph nodes. Positron emission tomography (PET) scans is also used in the diagnosis.
Lymph Node Biopsy : It is used to differentiate the other type of lymphatic tissue disorders from giant lymph node hyperplasia.

The treatment depends on the type of condition.

Localized : The localized lumps can be removed surgically. If surgery is not possible, then other treatment techniques such as medications or radiation technique can be used to shrink or destroy the lymph node.
Multicentric : Surgery may not be possible always for the multicentric type as many numbers of lymph nodes are involved in it. However, sometimes when the spleen is enlarged due to giant lymph node hyperplasia ten it can be removed by surgery to reduce the symptoms.

The treatment of multicentric type usually involves medications and other therapies to control the over growth of the cells. The treatment depends on the extent of the condition and whether the patient has HIV or HHV-8 or both.

Monoclonal Antibodies : If the patient does not have HIV or HHV-8 infection, then the initial treatment can be with a monoclonal antibody such as siltuximab. Monoclonal antibodies are used to block the action of IL-6 protein which is involved in cell overgrowth.
Corticosteroids : Corticosteroids are used to control inflammation of the lymph nodes.
Chemotherapy : It is usually suggested when the body does not responds to monoclonal antibodies or the patient has organ failure. Chemotherapy usually slows down the overgrowth of the lymphatic cells.
Thalidomide : It is an immune system modulator. It is very effective at inducing remission in giant lymph node hyperplasia. It blocks the action of IL-6 protein.
Antiviral drugs : These are used to suppress HHV-8 or HIV infections.

Giant lymph node hyperplasia is classified into two main types. They are:

Localized : It is the most common type and it affects only single group of lymph nodes. They are mostly observed in chest and abdomen. The enlarged lymph nodes in the chest cause breathing problems by pressing the trachea or small breathing tubes. The enlarged lymph nodes in the abdomen can cause pain, a feeling of fullness, or trouble eating. Sometimes they are formed in the neck, groin or under arm which are identified as lumps. The affected lymph nodes can be removed by surgery.
Multicentric : This type is more serious than localized and affects more than one group of lymph nodes. It can also affect the other organs that contain lymph nodes. Sometimes it may occur in patients with human immunodeficiency virus (HIV). This type can cause serious infections, fever, weight loss, fatigue, night sweats, nerve damage, weakness, and numbness. It weakens the immune system and can be a life-threatening. It can increase the risk of developing lymphomas.

Giant lymph node hyperplasia is also classified based on how the lymph nodes appear under the microscope. These are called microscopic subtypes.

Hyaline vascular Type : It is the most common type and it is localized. In very rare cases, it can be multicentric. It often have few symptoms.
Plasma cell Type : It is mostly a muticentric type and sometimes localized.
Mixed Subtype :occurs very less often and it shows the areas of both hyaline vascular and plasma cell types.
Plasmablastic Type :It is like the plasma cell type and is usually multicentric.