T-cell acute lymphoblastic leukemia/lymphoma (T-ALL/T-LBL) is commonly associated with Notch 1 mutations. There is limited data on the relationship between Notch l and NF- κ B expression and clinical features in LBL. We evaluated the expression of Notch l and NF- κ B in LBL using immunohistochemistry and analyzed their relationship with clinical characteristics, treatment results, and survival. From October 2000 to August 2008, 34 untreated patients with LBL were enrolled in the study. Median age was 11.8 years (range, 1 - 25 years). Twenty-five patients were diag nosed with T-LBL and 9 patients with B-LBL. Most patients received chemotherapy consisting of modified ALL-BFM- 90. Notch l showed high expression in 68% of T-LBL and low expression in 100% of B-LBL (p = 0.015). High expres sion of Notch l positively correlated with presence of a mediastinal mass but not with 5-year event free survival (EFS) in T-LBL. NF- κ B showed high expression in 65% of all patients with LBL, with no difference between T- and B-LBL. NF- κ B expression was higher in T-LBL patients with bulky disease and B symptom; it did not correlate with 5-year EFS in T-LBL. Expression of Notch 1 and NF- κ B strongly correlated (p = 0.014) in T-LBL. Notch 1 is highly ex - pressed in T-LBL. NF- κ B is highly expressed in all patients with LBL with no difference between T-LBL and B-LBL. Notch 1 expression was significantly associated with NF- κ B expression in T-LBL. Notch l and NF- κ B may play an im portant role in the development of T-LBL; further investigation is warranted.
KeywordsLymphoblastic LymphomaNotch lNF-κB
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