Background: Intravascular lymphoma is a rare disease with usually fatal outcome, characterized by the proliferation within the lumen of small blood vessels of neoplastic large lymphoid cells of B-cell origin. We report a case of intravascular lymphoma revealed by diffuse telangiectasia and cauda equina syndrome.
Case report: A 64-year-old Vietnamese woman presented with unexpected fever and weight loss. Three months after the onset of the first symptoms, an oedema appeared on the lower limbs and the trunk, followed by the eruption of diffuse superficial telangiectasia. Neurologic examination revealed a cauda equina syndrome. The diagnosis of intravascular B cell lymphoma was established on cutaneous and muscular biopsy specimen. A moderate hemophagocytic syndrome was observed, confirmed by bone marrow biopsy. Corticosteroid therapy was started, followed by combination chemotherapy yielding complete response. Six months later death occurred, without evidence of relapse of intravascular lymphoma.
Discussion: Clinical presentation of intravascular lymphoma is often confusing, mimicking systemic disease, with a predilection for skin and nervous system involvement. Diagnosis is difficult and often an autopsy finding. Prognosis is generally poor, but favourable responses to chemotherapy have been observed after early diagnosis and treatment. The pathogenesis of intravascular lymphoma remains unknown. Dysfunction of cell-endothelial interaction affecting adhesion molecules has been suspected. The implication of Epstein-Barr virus in intravascular lymphoma remains controversial.