Intravascular large B-cell lymphoma

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma characterized by the selective growth of lymphoma cells within the lumen of small blood vessels, particularly capillaries, and not in the lymph nodes. The disease affects adults and is often systemic at presentation, with symptoms related to organ ischemia or infarction.

Clinical Presentation

The clinical presentation of IVLBCL is highly variable and nonspecific, often leading to a delay in diagnosis. Symptoms can include fever, fatigue, and weight loss, as well as neurological symptoms, skin lesions, and abnormalities in liver function tests. The disease can affect any organ system, but the central nervous system and skin are most commonly involved.

Diagnosis

Diagnosis of IVLBCL is challenging due to its nonspecific symptoms and the absence of lymphadenopathy. The gold standard for diagnosis is a biopsy of an affected organ. Immunohistochemistry is used to identify the presence of large B cells within blood vessels.

Treatment

Treatment for IVLBCL typically involves aggressive chemotherapy, often with a regimen that includes rituximab, a monoclonal antibody that targets the CD20 protein on the surface of B cells. In some cases, high-dose chemotherapy followed by autologous stem cell transplantation may be considered.

Prognosis

The prognosis for IVLBCL is generally poor, with a median survival of less than one year in most series. However, early diagnosis and aggressive treatment can improve survival.

See Also

• Non-Hodgkin lymphoma
• Lymphoma
• Rituximab