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  • Küçük Resim
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    Different kinetics and risk factors for isolated extramedullary relapse after allogeneic hematopoietic stem cell transplantation in children with acute leukemia
    (Elsevier, 2021) Hazar, Volkan; Öztürk, Gülyüz; Yalçın, Koray; Uygun, Vedat; Aksoylar, Serap; Bozkurt, Ceyhun
    Relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the most frequent cause of posttransplant mortality. Isolated extramedullary relapse (iEMR) after HSCT is relatively rare and not well characterized, particularly in pediatric patients. We retrospectively analyzed 1527 consecutive pediatric patients with acute leukemia after allo-HSCT to study the incidence, risk factors and outcome of iEMR compared to systemic relapse. The 5-year cumulative incidence of systemic relapse ?either bone morrow (BM) only or combined with EMR? and iEMR was 24.8% and 5.5%, respectively. The onset of relapse after allo-HSCT was significantly longer in EM sites than in BM sites (7.19 and 5.58 months, respectively; p: 0.013). CR2+/active disease at transplantation (HR, 3.1, p<0001) and prior extramedullary disease (HR, 2.3; p: 0.007) were independent risk factors for iEMR. Chronic graft-versus-host disease (GVHD) reduced the risk of systemic relapse (HR, 0.5; p: 0.043) but did not protect against iEMR. The prognosis of patients who developed iEMR remained poor but was slightly better than that of patients who developed systemic relapse (3-year overall survival, 16.5% vs 15.3%, p: 0.089). Patients experiencing their first systemic relapse continued to have further systemic relapse, but only a minority progressed to iEMR, while those experiencing their iEMR at first relapse developed further systemic and isolated extramedullary relapses with approximately similar frequencies. Second iEMR was more common after the first iEMR than after the first systemic relapse (58.8% vs 13.0%, p: 0.001) and was associated with a poor outcome. iEMR has a poor prognosis, particularly after the 2nd relapse, and effective strategies are needed to improve outcomes.
  • Küçük Resim
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    Prognostic factors for survival in children who relapsed after allogeneic hematopoietic stem cell transplantation for acute leukemia
    (2020) Hazar, Volkan; Tezcan Karasu, Gülsün; Öztürk, Gülyüz; Küpesiz, Alphan; Aksoylar, Serap; Özbek, Namık; Uygun, Vedat; İleri, Talia; Okur, Fatma Visal; Koçak, Ülker; Çakı Kılıç, Suar; Akçay, Arzu; Güler, Elif; Kansoy, Savaş; Karakükcü, Musa; Bayram, İbrahim; Aksu , Tekin; Yeşilipek, Akif; Karagün, Barbaros Şahin; Yılmaz, Şebnem; Ertem, Mehmet; Uçkan, Duygu; Fışgın, Tunç; Gürsel, Orhan; Yaman, Yöntem; Bozkurt, Ceyhun; Gökçe, Müge
    Background: Post-transplant relapse has a dismal prognosis in children with acute leukemia undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Data on risk factors, treatment options, and outcomes are limited. Procedure: In this retrospective multicenter study in which a questionnaire was sent to all pediatric transplant centers reporting relapse after allo-HSCT for a cohort of 938 children with acute leukemia, we analyzed 255 children with relapse of acute leukemia after their first allo-HSCT. Results: The median interval from transplantation to relapse was 180 days, and the median follow-up from relapse to the last follow-up was 1844 days. The 3-year overall survival (OS) rate was 12.0%. The main cause of death was disease progression or subsequent relapse (82.6%). The majority of children received salvage treatment with curative intent without a second HSCT (67.8%), 22.0% of children underwent a second allo-HSCT, and 10.2% received palliative therapy. Isolated extramedullary relapse (hazard ratio (HR): 0.607, P = .011) and relapse earlier than 365 days post-transplantation (HR: 2.101, P < .001 for 0-180 days; HR: 1.522, P = .041 for 181-365 days) were found in multivariate analysis to be significant prognostic factors for outcome. The type of salvage therapy in chemosensitive relapse was identified as a significant prognostic factor for OS. Conclusion: A salvage approach with curative intent may be considered for patients with post-transplant relapse, even if they relapse in the first year post-transplantation. For sustainable remission, a second allo-HSCT may be recommended for patients who achieve complete remission after reinduction treatment.