Clinical spectrum and prognosis of follicular lymphoma with blastoid transformation: case series and a review of the literature
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Gayatri Vaidyanathan & Nuttapong Ngamphaiboon & Francisco J. Hernandez-Ilizaliturri
- چاپ و سال / کشور: 2011
Description
Follicular lymphoma (FL) with blastoid transformation (FL-BT) is a rare entity. FL-BT has a dismal prognosis due to intrinsic biological characteristics, lack of evidencebased therapeutic guidelines, and exclusion fromclinical trials. In order to understand the natural course of the disease and reconcile treatment strategies in patients with FL-BT, we reviewed available data. Thirty patients were identified, three from our institution (Roswell Park Cancer Institute) and 27 patients from peer-reviewed literature. Demographic, clinical, treatment, pathological, and cytogenetic characteristics were described. Most of the patients (90%) were treated with chemotherapy at transformation. Complete and partial responses to treatment were observed in 38.9% and 22.3% of the patients, respectively. The median overall survival for the entire group of patients was 11 months (range 0.8–30 months); half of the patients were found to have concurrent genetic alterations in the BCL-2 and C-MYC gene rearrangements (double hit). FL-BT with a double hit had a shorter median overall survival when compared to nondouble hit FL-BT (7 months vs. 26 months, respectively, P=0.005). In our experience, FL-BT is a rare and grave clonal evolution in FL characterized by an aggressive clinical course, inferior response to standard chemoimmunotherapy, and poor clinical outcome. C-MYC rearrangements are common in FL-BT and may play a role in the progression from FL to FL-BT or the poor clinical outcomes observed. Periodic updates from academic institutions treating FL-BT may improve patient care.
Ann Hematol (2011) 90:955–962 DOI 10.1007/s00277-011-1162-y Received: 17 September 2010 / Accepted: 17 January 2011 / Published online: 1 February 2011
