Intravascular T cell Lymphoma: A Rare, Poorly Characterized Entity with Cytotoxic Phenotype (P4.060)

Objective: To identify unique characteristics of intravascular T cell lymphoma that might provide insight into early diagnosis and treatment Background: Intravascular T-cell lymphomas are rare, poorly characterized lesions. Most cases are large B cell lymphoma with only a few cases of T cell/natural killer cell subtype. Intravascular lymphoma cases share a common feature of multi-organ involvement, poor prognosis, and EBV integration. Furthermore most cases reported as intravascular T cell or NK-cell lymphomas are mainly extranodal NK/T cell lymphoma, nasal type and anaplastic large cell lymphoma. We present the case of a 63 year old woman with a history of progressive central nervous system abnormalities. A brain biopsy identified an abnormal T-cell population confined to lumens of vessels. The lack of extranodal involvement, association with EBV, and multisystem organ involvement make this a unique type of intravascular T cell lymphoma that has yet to be described in the literature. Design/Methods: Morphologic pathologic examination of routine hematoxylin and eosin stains of brain biopsy specimen was performed as well as Chromogenic in-situ hybridization studies conducted with probes for Epstein-Barr virus-encoded small RNA . Molecular studies were conducted on DNA extracted from both the brain biopsy specimen and the bone marrow aspirate. Results: Initial brain MRI revealed two enhancing lesions in the left frontal operculum and right parietal cortex with associated restricted diffusion. Molecular studies showed a clonal T-cell population in both the brain and the bone marrow. In spite of therapy the patient deceased from rapid progression of disease. Conclusions: Treatment of CNS IVL has yet to be established due to rarity and highly aggressive course with poor response and short survival. In conclusion, this abnormal population of cytotoxic T-cells with intravascular localization probably represents a specific type of T-cell lymphoma and additional cases need to be identified, in the hope of institution of an early, appropriate therapy. Disclosure: Dr. Sharma has nothing to disclose. Dr. Yeaney has nothing to disclose. Dr. Soltanzadeh has nothing to disclose. Dr. Li has nothing to disclose. Dr. Cotta has nothing to disclose.