Abstract
Intravascular large B-cell lymphoma (IVLBCL) is a rare fatal neoplasm characterized by intravascular proliferation of lymphoma cells that commonly affects the central nervous system and skin. A 66-year-old woman, with a prior history of sudden onset of right hearing loss, developed transient monoplegia in the left arm in November 2008. Warfarin was started because of the patent foramen oval and stagnation in the deep vein of her legs. Since then, she developed various neurological episodes including brain stem, cranial nerves, and myelopathic abnormalities as well as skin rash, fever and hematologic abnormalities with a high serum LDH, monocytosis, anemia, thrombocytopenia, and splenomegaly. A skin biopsy and bone marrow aspiration revealed no atypical cells. Because she presented with high fever and body weight loss, low-dose prednisolone was started and warfarin was stopped. But even with steroid initiation, she became confused. Then, diffusion and FLAIR MR images showed multiple small hyperintense lesions in the cortical and subcortical regions. A stereotactic open brain biopsy of the left frontal cortex revealed large atypical intravascular tumor cells occluding the lumen of small cortical and meningeal vessels, and immunohistochemical staining demonstrated tumor cells as B cell-origin. She received R-CHOP chemotherapy and achieved a partial remission. IVL should be considered as an important differential diagnosis for patients presenting with a variety of neurological disorders and skin lesions accompanied by a high serum LDH. While brain biopsy is a rather invasive measure, in some cases, it can be only a diagnostic clue.
