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Fakhfakh, Y.1; Frikha, I.1; Sannenna, H.2; Mnif, S.3; Chaari, M.4; Hdiji, S.1; Mdhaffar, M.1; Kassar, O.1; Ghorbel, M.1; Charfi, M.1; Saad, A.2; Benothmen, T.5; Elleuch, H.4; Elloumi, M.1

doi: 10.1097/01.HS9.0000565240.15881.a9
Publication Only: Acute lymphoblastic leukemia - Clinical

1hematology, university of Sfax, Sfax

2cytogenetic, university of Sousse, sousse

3hematobiology, university of Tunis, tunis

4hematobiology, university of Sfax, Sfax

5hematology, university of Tunis, tunis, Tunisia

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Since the era of therapy targeted by tyrosine kinase inhibitors (TKIs), the prognosis of acute lymphoblastic leukemia (ALL) in adult Philadelphia chromosome positive (Ph +) has been significantly improved.

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We evaluate in this study the outcome of adult Ph + ALL treated with TKI associated with chemotherapy.

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Our study is retrospective, including cases of de novo Ph + adult ALL diagnosed in the Hematology Department of Hedi Chaker Hospital of Sfax from January 2009 to March 2018. Cytogenetic analysis and research Bcr-Abl transcript was performed for all patients. They were treated according to the GRAAPH 2005 protocol. TKI, Imatinib, was used at a dose of 600 to 800 mg/day. Bone marrow allograft has been indicated in patients younger than 50 years, in complete remission and with an HLA compliant intra-familial donor. The current study reports the clinical features and treatment outcome of patients with Ph + ALL.

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There were 13 patients diagnosed with Ph + ALL among 51 with ALL (25%). The median age at diagnosis was 44 years (range 20-54 years) and the sex ratio was 0.62. The presence of t (9.22) was found in 8 cases, for all patients with a conclusive karyotype, 5 of them had additional abnormalities. Twelve had a positive Bcr-Abl transcript. One patient had a positive Bcr-Abl transcript but the cytogenetic study was not concluded. Complete remission (CR) was noted in 92% of cases. Eight patients (age <50 years) were on allograft indications, seven of them had a compatible HLA donor. Of these, five patients had an allograft: four patients are alive in major molecular response after a mean follow-up of 5 years (1-7 years). One patient died in post-transplant by acute GVH. For the 8 patients who did not have an allograft, 4 unfortunately died, the 4 others are alive in major molecular response after a mean follow-up of 4 years (1-7 years).

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Before the TKI era, adult Ph + ALL had a poor prognosis with unsatisfactory CR and survivals not exceeding 40%. Allogenic stem cell transplantation remains the only curative option for patients with suitable matched donors. Since the introduction of TKIs in chemotherapy regimens, the prognosis has been much improved, leading to a CR rate exceeding 80% in the literature, and an event-free 5-year survival rate exceeding 70%t, this was also found in our series.

Copyright © 2019 The Authors. Published by Wolters Kluwer Health Inc., on behalf of the European Hematology Association.