Intravascular lymphoma (IVL) is an aggressive (fast-growing) cancer that forms in the blood vessels. This cancer is classified as type of non-Hodgkin lymphoma, one of the two main categories of lymphoma. It generally develops from a type of white blood cell called B lymphocytes, or B-cells. Less commonly, IVL can develop from other types of white blood cells called T lymphocytes (T cells) or natural killer cells.

A very rare cancer, IVL is most commonly diagnosed in adults older than 50. Intravascular lymphoma is also known by many other names, including:

• Intravascular lymphomatosis
• Intravascular large B-cell lymphoma
• Angiotropic large cell lymphoma
• Malignant angioendotheliomatosis

What Causes Intravascular Lymphoma?

People develop IVL due to genetic abnormalities that lead to a proliferation (growth) of cells inside the lumina (hollow centers) of blood vessels, particularly in small blood vessels like capillaries. Large tumor cells accumulate inside these vessels, causing several complications.

Symptoms of Intravascular Lymphoma

On the whole, IVL can cause a variety of signs and symptoms due to widespread organ involvement. Common symptoms of IVL include:

• Fever, unexplained weight loss, and night sweats (categorized as B symptoms)
• Painful skin lesions
• Neurological problems, such as confusion and stroke
• Memory loss
• Spider veins
• Organ failure

Symptoms can vary significantly from person to person, and by the type of IVL. Enlargement of the lymph nodes (lymphadenopathy) is typically not observed in people with IVL, which can make it difficult to diagnose.

Types of Intravascular Lymphoma

There are three subtypes of IVL that affect different parts of the body. These subtypes tend to occur in different parts of the world.

Classical Variant

The classical variant of IVL occurs more frequently in western countries. This type often affects a person’s skin and their central nervous system (CNS), which includes the brain and spinal cord.

Hemophagocytic Syndrome-Associated Variant

This type is also called the Asian variant, due to its higher prevalence in Asian countries. It’s associated with hemophagocytic syndrome, a condition that affects the CNS, liver, and spleen.

Cutaneous Variant

The cutaneous variant of IVL primarily affects a person’s skin and causes one or multiple skin lesions or tumors. This variant is diagnosed much more frequently in women. It can be less aggressive than the other two variants.

How Is Intravascular Lymphoma Diagnosed?

Diagnosis of IVL involves several medical procedures and tests.

Skin Biopsy

A skin biopsy, which involves collecting a tissue sample from the skin, is the primary procedure used to diagnose IVL. A biopsy may be either a deep skin biopsy of a skin lesion or a random biopsy of normal-looking skin. Medical personnel can then analyze the tissue sample under a microscope and confirm the presence of lymphoma cells.

Bone Marrow Testing

A doctor may require a bone marrow biopsy or aspiration to determine if there are cancer cells in someone’s bone marrow (the spongy tissue found inside certain bones). Bone marrow involvement is common in IVL, particularly in the hemophagocytic variant.

Imaging Tests

Imaging technologies used to view tissues inside the body may include:

• Magnetic resonance imaging (MRI)
• Computed tomography (CT) scans
• Positron emission tomography combined with CT (PET-CT)

An MRI may be used to see CNS involvement, which commonly occurs in IVL. Both CT and PET-CT can detect hepatosplenomegaly (enlargement of the liver and spleen), a common finding in the hemophagocytic syndrome-associated variant. Further, PET-CT may also help to see other internal lesions.

Blood Tests

Your doctor may use blood tests to look for signs of IVL by measuring blood cell counts and protein levels. Low levels of white blood cells, red blood cells, and platelets are often caused by IVL. Additionally, a person with IVL may have high levels of certain proteins such as lactate dehydrogenase protein and beta-2 microglobulin.

Lumbar Puncture

A lumbar puncture (spinal tap) is a technique in which a doctor uses a needle to collect cerebrospinal fluid from the spinal canal. This procedure allows your doctor to look for evidence of lymphoma in the CNS.

Treatments for Intravascular Lymphoma

Because IVL is so rare, there are no drugs or treatment regimens designed specifically to treat the disease. Rather, treatment options for IVL include therapies used for other forms of lymphomas that affect the CNS.

Treatment for intravascular lymphoma typically involves chemotherapy together with an antibody prescription drug called Rituxan (rituximab). Rituximab recognizes a specific molecule, called CD20, that is present on the surface of lymphoma cells.

One chemotherapy treatment regimen used to treat IVL is known as R-CHOP, which consists of rituximab plus a combination of the chemotherapeutic drugs:

• Cytoxan (cyclophosphamide)
• Doxorubicin (also known as hydroxydaunorubicin and sold as Adriamycin)
• Vincristine sulfate (formerly sold as Oncovin)
• Prednisone (a corticosteroid)

Normal chemotherapy treatment doesn’t effectively reach the CNS, so a doctor may treat a person with IVL using intrathecal chemotherapy. This procedure administers chemotherapy (usually the drug methotrexate) directly to the CNS with a lumbar puncture.

Intrathecal chemotherapy may be given as the initial treatment in a person whose CNS is already affected, or as CNS prophylaxis (prevention) for a person whose CNS is not yet affected. CNS prophylaxis allows a person to be treated before cancer spreads to the CNS to prevent a more serious condition. Methotrexate can also be administered via a catheter inserted into the brain.

Radiation therapy to the brain might also be given following treatment with methotrexate.

Prognosis for Intravascular Lymphoma

Due to the aggressive nature of the disease and widespread organ involvement, IVL has a poor prognosis. People with IVL usually live for a few months after diagnosis. Much knowledge of the disease comes from case reports that follow just a single person, making IVL and its prognosis difficult to study. Additional research is needed to improve diagnostic approaches and identify more effective treatments for IVL.

Talk With Others Who Understand

On MyLymphomaTeam, the social network for people with lymphoma and their loved ones, more than 10,000 members understand what it’s like to face lymphoma and can provide you with support and answers.

Are you or a loved one newly diagnosed with intravascular lymphoma? Share your experience in the comments below, or start a conversation by posting on MyLymphomaTeam.