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Non-Hodgkin lymphoma

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Intravascular large B-cell lymphoma

Intravascular large B-cell lymphoma is a very rare subtype of diffuse large B-cell lymphoma (DLBCL). It is also considered a distinct type of non-Hodgkin lymphoma (NHL) in the World Health Organization (WHO) classification system. Intravascular large B-cell lymphoma affects small blood vessels. With this B-cell type of lymphoma, cancer cells circulate in the blood and block (occlude) small blood vessels. Intravascular large B-cell lymphoma may also be called intravascular lymphomatosis or malignant angioendotheliomatosis.

Intravascular large B-cell lymphoma usually affects the small blood vessels in many organs, including:

  • central nervous systemcentral nervous systemThe brain and spinal cord, which work together to control all the functions of the body. (CNS)
  • kidneys
  • lungs
  • skin

However, almost any site can be affected.

People with intravascular large B-cell lymphoma have a variety of symptoms. The symptoms will depend on the tissue or organ affected when the blood vessels become occluded. This type of lymphoma is often difficult to diagnose because the symptoms can be so varied.

Intravascular large B-cell lymphoma is a fast-growing (aggressive) lymphoma. People with this type of lymphoma often have a poor prognosis.

Treatment

Treatment of intravascular large B-cell lymphoma often involves chemotherapy.

Chemotherapy

A combination of chemotherapy drugs is usually used to treat intravascular large B-cell lymphoma. The same combination used to treat DLBCL is often used.

  • CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone (Deltasone)

Biological therapy

Rituximab (Rituxan) is a type of biological therapy called a monoclonal antibody. Monoclonal antibodies are effective in treating certain types of NHL. Rituximab is usually used with chemotherapy to treat intravascular large B-cell lymphoma.

For more detailed information on specific drugs, go to sources of drug information.

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