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Intravascular large B-cell lymphoma
Intravascular large B-cell lymphoma (ILCL) may also be called intravascular angiotropic lymphoma. It is a very rare, fast-growing (aggressive) subtype of diffuse large B-cell lymphoma (DLBCL).
ILCL usually occurs in middle-aged or elderly people. It develops slightly more often in men than in women.
With ILCL, the lymphoma cells circulate in the blood and can block small blood vessels. It usually affects the small blood vessels in many organs such as the brain and spinal cord (together called the central nervous system, or CNS), kidneys, lungs and skin.
ILCL is often difficult to diagnose because the symptoms can be so varied. The symptoms depend on the tissue or organ affected when the blood vessels become blocked.
Most people have B symptoms, which are unexplained fever, drenching night sweats and unexplained weight loss. ILCL progresses quickly and can cause peripheral neuropathy, dementia and stroke. Some people will develop a skin rash.
ILCL is usually treated with chemotherapy and targeted therapy.
A combination of chemotherapy drugs is usually given to treat ILCL. A common combination used is CHOP, which is:
- cyclophosphamide (Cytoxan, Procytox)
- doxorubicin (Adriamycin)
- vincristine (Oncovin)
Central nervous system prophylaxis or treatment
ILCL can spread to the CNS. CNS prophylaxis is used to prevent lymphoma cells from entering the brain and spinal cord. Treatments are also given if precursor lymphoblastic lymphoma has spread to the CNS.
CNS prophylaxis or treatment may be given as intrathecal chemotherapy. This means that the chemotherapy drug is injected directly into the cerebrospinal fluid (CSF). The drug used in intrathecal chemotherapy is often methotrexate or cytarabine.
CNS prophylaxis or treatment can also be given by a needle in a vein, or intravenously. The drug used for intravenous chemotherapy is high-dose methotrexate.
Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
A targeted therapy drug used along with chemotherapy to treat ILCL is rituximab. R-CHOP is CHOP with rituximab (Rituxan).
People with ILCL that doesn’t go away with treatment or that comes back after other treatments may be offered immunotherapy called CAR T-cell therapy.
CAR T-cell therapy takes millions of T cells from a person with cancer. In the lab, they are changed so they have chimeric antigen receptors (CARs) on their surface. These receptors recognize a specific antigen (protein) found on the type of cancer being treated. The T cells are then given back to the person where they multiply, attack and destroy the cancer cells.
Axicabtagene ciloleucel (Yescarta) is a CAR T-cell therapy approved to treat people with high grade B-cell lymphomas that have relapsed (come back after treatment) or are refractory (not responded to treatment) after at least 2 other treatments.
A disorder of the peripheral nerves (nerves outside the brain and spinal cord) that causes pain, numbness, tingling, burning, swelling, muscle weakness and loss of reflexes in different parts of the body.
Peripheral neuropathy may be caused by physical injury, infection, toxic substances or disease (such as cancer, diabetes, kidney failure or malnutrition). It can also be a side effect of some cancer treatments, including chemotherapy and radiation therapy.