الفهرس | Only 14 pages are availabe for public view |
Abstract Background: ALL is the most common form of pediatric malignancy. B-cell ALL tends to be more common in Hispanic populations; however, most other forms of ALL are more common in non-Hispanic white men. Aim of the Work: The aim is to detect the incidence and the prognostic outcome of CRLF2 overexppresion in ph- negative ALL and evaluate the response of this group of patients regarding response rate, overall survival, progression free survival and relapse rate. Patients and Methods: To evaluate the prognostic importance of CRLF2 rearrangement, 84 Philadelphia negative adult ALL patients from Ain Shams University hospital and Nasser Institute Hospital were included in this study during the period from May 2017 to April 2019, with a follow-up period of at least 12 months. The patients received chemotherapy according to each patient’s age and comorbidities. The chemotherapeutic regimens included Hyper CVAD, DFCI, Hoelzer’s, and Total XVI. Results: Our study shows a high frequency of Ph-like ALL in adults and significantly worse outcomes in the CRLF2+ subset of Ph-like ALL. Patients with CRLF2 rearrangement tended to be older and with higher WBC counts, lower hemoglobin and platelet counts at presentation than non-CRLF2 ALL. Patients with Ph-like ALL also had significantly worse OS and DFS compared to patients without Ph-like ALL (2-year survival: 26.7% vs. 58%, P = 0.028). This finding is similar to those reported by other authors. Conclusion: The biologic heterogeneity of this disease leads to challenges not only in diagnosis but also in the investigation of novel therapies. There is an immense potential for targeted therapeutic strategies involving kinase inhibitors as well as immune therapies targeting the CRLF2 molecule on the cell surface. Novel strategies are needed to improve the outcome of these patients |