Castleman disease


Castleman disease

Description, Causes and Risk Factors:

Castleman disease is a rate and usually benign condition of unknown etiology resulting in extensive lymphoid proliferation. Castleman disease is separated into 2 groups: localized/unicentric and multicentric/systemic. Localized/unicentric Castleman disease affects only a single lymph node (or lymph node group). The multicentric/systemic type affects 2 or more groups of lymph nodes in different parts of the body. It may also involve internal organs like the spleen or liver.

Castleman disease

The exact cause of Castleman disease remains unknown. Infection by a virus called the human herpes virus 8 (HHV-8) is associated with Castleman disease, and doctors suspect that it may play a causal role, especially in multicentric Castleman disease. This virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor of the blood vessel walls, which is also common in people with multicentric Castleman disease. People who are HIV-positive are more likely to have both Castleman disease and Kaposi sarcoma.

Researchers are not clear what the precise role of HHV-8 may be, though it appears that it may help malfunctioning immune system cells reproduce rapidly. The immune system cells are called IL-6 (interleukin-6), and a protein produced by these cells may contribute to the overgrowth of lymphatic cells.

The HHV virus is likely transmitted through saliva, but may also be passed from person-to-person via sexual contact, blood transfusions and transplants.

Generally, unicentric Castleman disease isn't associated with HHV-8.

Symptoms:

Symptoms may include:

    A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating.

  • Low-grade fever.

  • Weight loss.

  • Skin rash.

  • Fatigue.

  • Excessive sweating.

  • Anemia.

  • Night sweats.

  • Loss of appetite.

  • Nausea and vomiting

  • Enlarged peripheral lymph nodes, usually around the neck, collarbone, underarm and groin areas.

  • Enlarged liver or spleen

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

Diagnosis:

Differential Diagnosis:Reactive lymph node hyperplasia, malignant lymph node hyperplasia.

The diagnosis of Castleman's disease is based on histological examination of the lesion with immunohistochemical labeling. Castleman's disease is defined as angiofollicular lymphoid hyperplasia, which can present in different ways. The hyaline-vascular form, the most common is often observed in localized forms. The plasmacytic form, the rarest, is mostly seen in multicentric forms. Finally, an intermediary or mixed form has also been described. The lymphocyte proliferation is usually polyclonal. However, monoclonal proliferation and rearrangement of immunoglobulin and/or T-cell receptor genes have been reported in certain patients with multicentric Castleman's disease.

Other Tests May Include:

    Physical examination: Your doctor may examine not only your swollen lymph nodes but also your other lymph nodes to determine their size and consistency.

  • Blood and urine tests: Blood and urine tests may help your doctor rule out other infections or diseases. They can also reveal anemia and abnormalities in blood proteins that are sometimes characteristic of Castleman disease.

  • Imaging techniques: An X-ray, computerized tomography (CT) scan or magnetic resonance imaging (MRI) scan of your chest, neck, abdomen and pelvis may detect the presence and number of enlarged lymph nodes. These tests can also determine whether organs, such as your liver or spleen, are enlarged. Positron emission tomography (PET) scans also may be used in diagnosing Castleman disease and later, to assess whether a treatment is working.

  • Lymph node biopsy: To differentiate Castleman disease from other types of lymphatic tissue disorders, such as lymphoma, it's necessary to take a sample of lymph node tissue for examination in the laboratory. If the enlarged lymph node is close to the surface of your skin, the biopsy can be done under local anesthesia. If it's in your chest or abdomen, more extensive surgery may be necessary to access the lymph node. By looking at the structure of the cells, a doctor who specializes in diagnosing disease (pathologist) may be able to tell whether it's lymphoma or Castleman disease.

  • Immunohistochemistry: In this test, a part of the biopsy sample is treated with special man-made antibodies. The cells are treated so that certain types of cells change color. The color change can be seen under a microscope. It may help tell whether there is CD or lymphoma in the lymph node.

  • Flow cytometry: Cells from the lymph node are treated with special manmade antibodies and passed in front of a laser beam. Each antibody sticks only to certain types of cells. If the sample contains those cells, the laser light causes them to give off light of a different color. The intensity of each color is measured exactly and analyzed by a computer. This test can help determine whether lymph node swelling is caused by lymphoma, some other cancer, or a non-cancerous disease like Castleman disease.

Treatment:

The best treatment for localized forms of Castleman's disease is complete surgical excision. This treatment allows full recovery without relapse in almost all cases.

Treating multicentric Castleman disease is generally more difficult. Because the disease is rare, has varied nonspecific signs and symptoms, and spontaneously goes into remission at times, doctors have found it difficult to identify the best treatment. In addition, there are no clinical trials that offer definitive evidence in favor of any therapy. It's also not clear if treatment should differ based on HIV status.

Most therapies are palliative, which means their goal is to relieve signs and symptoms rather than cure the illness. Surgery usually isn't an option for multicentric disease because of the number of lymph nodes involved, although sometimes removing an enlarged spleen may help ease symptoms.

Therapies that are commonly used for multicentric disease, with varying degrees of success, include the following. Doctors generally try as many therapies as they can to provide the most relief which may include corticosteroids, chemotherapy, antiviral drugs, monoclonal antibodies, immune modulators.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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