What Is Intravascular Lymphoma?

Intravascular lymphoma is a subtype of 'Lymphoma', an umbrella term that loosely refers to several dozen independent categorical types and subtypes of cancers of the lymphatic system. It is also known as intravascular large B-cell lymphoma and was previously known as angiotropic lymphoma.

Intravascular lymphoma (IVL) is a very rare sub-subtype of a very common subtype of non-Hodgkin's lymphoma, extranodal diffuse large B-cell lymphoma. What happens in IVL is that cancerous lymphocytes, which are distorted in shape and size compared to healthy lymphocytes, will pile up in the associated blood vessels, causing swelling and distention.

Affecting patients at middle-age and older, as an extranodal disease Intravascular lymphoma will affect organs and this disease can affect virtually any organ in the body. Unlike most lymphomas, intravascular lymphoma does not commonly involve the lymph nodes. This also holds true for the spleen.

While the vast majority of cases of intravascular lymphoma are of B-cell lineage, rarely the disease will affect the T-cells.

Symptoms of intravascular lymphoma

Because IVL is an extranodal disease, this means that symptoms will vary, depending on the extranodal organ that is being affected. Generalized symptoms may include fatigue, but perhaps the key clinical feature of IVL is vascular obstruction and subsequent edema depending on where this is occurring and what organ(s) is affected.

The extranodal sites most frequently involved in IVL are the skin and the central nervous system, but it has also been known to affect the kidneys, lungs, and GI tract, among other organs.

Diagnosing intravascular Lymphoma

IVL is notorious for presenting itself in various and misleading ways, making diagnosis extremely difficult, especially since it is so rare that doctors are not typically looking for it. Consequently, until recently a diagnosis of intravascular lymphoma has most often been made post-mortem, after the patient has already died.

However, as pathology techniques have improved, along with imaging techniques, the diagnosis of intravascular lymphoma is being made more and more often before the patient dies and in time to offer therapeutic intervention.

Treatment and prognosis of intravascular lymphoma

Because it has traditionally been overlooked or diagnosed too late, and because of its aggressive nature, the mortality rate of intravascular lymphoma is very high. When the disease is diagnosed early, therapeutic intervention with chemotherapy has shown to be successful, even curative in some cases.

Sources

• Zuckerman D et al. Intravascular Lymphoma: The Oncologist's 'Great Imitator'. The Oncologist, Vol. 11, No. 5, 496-502.
• Ferry JA. Extranodal Lymphoma. Archives of Pathology & Laboratory Medicine: Vol. 132, No. 4, pp. 565-578.

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