Intravascular lymphomas

Intravascular lymphomas are a rare subtype of extranodal lymphoma that are characterized by the selective growth of lymphoma cells within the lumen of blood vessels, particularly the capillaries, rather than in the lymph nodes. The disease affects adults and is usually systemic at diagnosis, with a predilection for the central nervous system and skin.

Clinical Presentation

The clinical presentation of intravascular lymphomas is often nonspecific and can vary widely, depending on the organs involved. Symptoms may include fever, fatigue, weight loss, neurologic symptoms, and skin lesions. Because of the nonspecific symptoms and the rarity of the disease, diagnosis is often delayed.

Diagnosis

Diagnosis of intravascular lymphomas is challenging due to the lack of specific symptoms and the absence of lymphadenopathy or hepatosplenomegaly. The diagnosis is often made by biopsy of involved organs or tissues. Immunohistochemistry is used to confirm the diagnosis, with the lymphoma cells typically expressing CD20, a B-cell marker.

Treatment

Treatment of intravascular lymphomas typically involves chemotherapy, often with a regimen that includes rituximab, a monoclonal antibody against CD20. The prognosis is generally poor, with a median survival of less than two years, but early diagnosis and aggressive treatment can improve survival.

See Also

• Lymphoma
• Extranodal lymphoma
• CD20
• Rituximab

References

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