Intravascular B-cell lymphoma in a 38-year-old woman: a case report

Abstract.

Intravascular lymphoma (IVL) is a rare aggressive disease characterised by the presence of lymphoma cells only in the lumina of small vessels, particularly capillaries. Only about 200 cases have been reported in the world (some of them retrospectively). IVL is predominantly of B-cell lineage origin but occasionally T-cell lineage occurs. Multiple organs may be involved and a variety of clinical presentations have been described. These include nephrotic syndrome, pyrexia and hypertension, breathlessness and haemolytic anaemia, leukopoenia, pancytopoenia and disseminated intravascular coagulation. We report a case of a 38-year-old woman with a highly aggressive clinical course of IVL. She was admitted to the Department of Neurosurgery because of spondylolisthesis of L5-S1 qualified to surgery. During hospitalisation haemolytic anaemia, thrombocytopoenia and splenomegaly were observed and she was admitted to the Department of Haematology for diagnosis. During her staying in the hospital, new symptoms, such as kidney and liver failure, occurred and the central nervous system was involved. The clinical course was very rapid and progressive. Corticosteroid therapy was started but the disease soon led to the fatal outcome. Diagnosis was established at post-mortem examination.