Abstract 33: Epidemiology and clinicopathology of T-cell intravascular lymphoma (TIVL): Making the case for extra-nodal NK-T cell lymphoma-angiotropic type as a provisional entity

• Published in: Cancer research (Chicago, Ill.) Ill.), 2020-08, Vol.80 (16_Supplement), p.33-33
• Main Authors: Haddad, Philip A., Gallagher, Kevin, Hammoud, Dalia
• Format: Article
• Language: English
• ISSN: 0008-5472; 1538-7445
• DOI: 10.1158/1538-7445.AM2020-33

TIVL is a rare and often aggressive NK/T-cell lymphoma characterized by the presence of neoplastic lymphocytes that are restricted to the lumen of small vessels. It comprises 8-10% of all IVL. By the time a diagnosis is made, TIVL tends to be widely disseminated across multiple extra-nodal sites with frequent catastrophic consequences. In order to study the demographic characteristics, presentation and progression patterns, survival, and prognostic factors, we constructed a database that pooled cases between 1986 and 2019. Kaplan-Meier survival curves were constructed. Cox proportional-hazards model and Log-rank tests were used to assess the influence of demographic and clinical factors on survival. A total of 81 patients with confirmed TIVL were identified. The median age was 53 (17-87) years with a peak incidence between ages 50 and 60. There was a slight male predominance with a male to female ratio of 1.5. Comprising 43% of reported cases, Asians were the most afflicted ethnic group. Skin (71%) was the most common site involved with TIVL followed by CNS (23%), and pulmonary and bone marrow involvement (8% each). The median overall survival of the whole group was 7 months. Sex and age did not seem to affect survival rates. When compared to supportive care, chemotherapy significantly improved survival (HR:0.15, p