251. Intravascular B-cell lymphoma: report of two cases with different clinical presentation but rapid central nervous system involvement.
- Author
-
Anghel G, Petrinato G, Severino A, Remotti D, Insabato L, De Renzo A, Rotoli B, and Majolino I
- Subjects
- Autopsy, Fatal Outcome, Female, Humans, Leg blood supply, Lymphoma, B-Cell diagnosis, Lymphoma, B-Cell therapy, Male, Middle Aged, Neoplasm Invasiveness pathology, Vascular Neoplasms diagnosis, Vascular Neoplasms therapy, Central Nervous System Neoplasms secondary, Lymphoma, B-Cell pathology, Vascular Neoplasms pathology
- Abstract
Intravascular lymphomatosis (IVL) is a rare large-cell lymphoproliferative disorder characterized by a widespread lymphoma proliferation within the lumen of medium and small vessels, frequently presenting with skin and/or central nervous system (CNS) manifestations. The tumor is of B-cell origin in most cases. Prognosis is poor with a reported median survival of 5-7 months. We describe here two cases of IVL. The first was that of a 55-year-old woman with a large B-cell lymphoma of the leg, successfully treated with conventional chemotherapy (CHT) followed by autologous peripheral stem cell transplantation. At 3 months from the autograft she relapsed with a picture of hemophagocytic syndrome (HPS) and CNS symptoms. She died before any specific treatment, and post-mortem examination revealed the intravascular proliferation of lymphoma B-cells in the brain and bone marrow. The second case was that of a 60-year-old male with CNS involvement at diagnosis. He responded poorly to CHOP-like CHT, and died 2 months after diagnosis and 6 months after onset of symptoms. Failure of CHT at least in some IVL patients may be related to a delay in the initiation of therapy due to non-specific neurological symptoms. Therefore, early diagnosis based upon aggressive attempts immediately followed by adequate therapy may prove beneficial to these patients. In the present report, we performed an extended medline-based review of the published series of patients with IVL.
- Published
- 2003
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