Intravascular lymphoma

Rare form of non-Hodgkin’s lymphoma known as angioendotheliomatosis or angiotropic large cell lymphoma.

Incidence is only 0.095 per 1,000,000.

Subtype of extranodal diffuse B cell lymphoma.

Also known as intravascular large B-cell lymphoma and was previously known as angiotropic lymphoma.

Characterized by selected growth of the tumor cells within blood vessel lumina.
Typically involves small blood vessels, particular capillaries and post capillary venules, usually sparing the lymphatic vessels.

A very rare sub-subtype of a very common subtype of non-Hodgkin’s lymphoma, extranodal diffuse large B-cell lymphoma.

The malignancy has a peculiar growth pattern with a virtual absence of masses.
Diagnosis is often achieved only on post Mortem examination.

Malignant lymphocytes, distorted in shape and size accumulate in the associated blood vessels, causing swelling and distention.

Affects patients at middle-age and older.

As an extranodal disease, affects virtually any organ in the body.

Does not commonly involve lymph node or splenic involvement.

Majority of cases are of B-cell lineage, but rarely affects the T-cells.

Symptoms of intravascular lymphomas vary, depending on the extranodal organ that is being affected.

The primary clinical feature of IVL is vascular obstruction and subsequent edema.

The extranodal sites most frequently involved are the skin and the central nervous system.

The most common manifestations involve skin lesions and constitutional symptoms, but these are present only in 40 and 50% of cases, respectively.
rates of CNS infiltration it’s estimated to be anywhere between 25-76% and focal neurological deficits, altered mental status, sensory motor deficits, neuropathies or seizures have been documented in 35% of cases.
Two forms exist: IVLBCL which affects almost all patients in European and North American countries and the hemophagocytic syndrome form which is more aggressive in frequently diagnosed in Japan.

May affect the kidneys, lungs, and GI tract.

Diagnosis extremely difficult.

Laboratory abnormalities are variable and non-specific.

Laboratory findings include anemia, elevated LDH, and elevated sedimentation rate.

Mortality rate is very high, but when diagnosed early, chemotherapy has shown to be successful, even curative in some cases.

Mature B cells identified, but T cells and natural killer cells have been described.

A systemic disease, typically occurs in elderly.

Most commonly diagnosed on postmortem examination.

Characterized by multifocal proliferation of pleomorphic large lymphoid cells within medium to small vessels.

Vessel walls and surrounding tissues that are uninvolved are spared.

Lymphadenopathy usually absent.

May originate from post-germinal center cells.

Rapidly progressive and fatal with lymphoma cells in the lumen of small blood vessels.

Incidence one in 1 million persons.

Mean age at diagnosis 70 years.

Majority of patients have CNS and skin involvement in Western countries and had a hemophagocytic syndrome in Asian countries.

Patients with central nervous system involvement may have neurologic abnormalities, generalized weakness, seizures, and altered mental status.

55-85% present wi B symptoms of fever, night sweats and weight loss attributed to small blood vessel occlusion of affected organs.

CNS involvement can be with focal neurologic abnormalities, altered mental status, or progressive dementia.

No diagnostic laboratory studies exist, but patients usually present with anemia, elevated LVH, and elevated sedimentation rate.

Associated with elevated B2 microglobulin, and IL2.

With CNS involvement CSF shows mildly increased leukocyte count and protein levels.

CSF cytology and flow cytometry usually negative in CNS involvement.

Diagnosis by biopsy skin, bone marrow or CNS with findings of intravascular B cells.

Typically in skin subcutaneous tissue fat.

Anthracycline-based chemotherapy with Rituxan is suggested treatment, although autologous cells stem cell transplant should be considered.

With CHOP-R three year survival ranges from 53-81%.

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