Intravascular large B-cell lymphoma: representative cases and approach to diagnosis

Intravascular large B-cell lymphoma (ILBCL) is a subtype of non-Hodgkin's large B-cell lymphoma that is characterised by neoplastic lymphocyte proliferation within the lumen of small blood vessels, which may occur without an extracellular tumour mass or peripheral blood involvement. This report highlights some of the diagnostic issues for ILBCL, and how it can be approached. The two cases described below highlight two significantly different presentations, one with predominately neurological phenomena, and the other with fever of unknown origin for investigation. Both patients were managed with chemotherapy and intercalated intrathecal chemotherapy, with good clinical outcomes, without further evidence of clinical relapse. These cases along with a review of the literature highlight the key learning points in the difficulties in the diagnosis of this condition, and the appropriate use of random skin biopsy in patient suspected of having ILBCL, such as those with constitutional symptoms with otherwise negative malignancy screening, and unexplained neurological phenomena, especially if recurrent in nature.