Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal large B-cell lymphoma. Most patients show non-specific symptoms including fever, elevated lactate dehydrogenase (LDH) and thrombocytopenia. IVLBCL tumor cells are frequently hard to detect because of the selective growth of lymphoma cells in the lumina of small vessels without apparent mass lesions. We experienced 2 cases diagnosed as IVLBCL by random skin biopsy, who had no visible skin lesions. One had suffered from left partial hemiparalysis and was initially diagnosed with diffuse large B-cell lymphoma (DLBCL) of the central nervous system. However, her condition worsened not withstanding whole brain radiation therapy for DLBCL. The other patient was thought to suffer from a collagen disease. She too had shown a poor response to high doses of corticosteroid for fever and severe thrombocytopenia. Blood examination revealed severe thrombocytopenia and increased serum LDH levels in both cases. Since we suspected these patients to be suffering from IVLBCL, random skin biopsies were performed, although no skin lesions were present in either case. Large B cell lymphoma cells in the venules were recognized in the specimens, resulting in the diagnosis of IVLBCL.
Random skin biopsy is a safe and useful diagnostic tool in patients with IVLBCL and should be performed in patients who are suspected of having this disease.