scholarly journals Treatment of PCM1-JAK2 fusion tyrosine kinase gene-related acute lymphoblastic leukemia with stem cell transplantation

2021 ◽  
pp. FRD10
Author(s):  
Henry G Kaplan ◽  
Carlo B Bifulco ◽  
Ruyun Jin ◽  
James M Scanlan ◽  
David Corwin
Keyword(s):  
Stem Cell ◽  
Acute Lymphoblastic Leukemia ◽  
Stem Cell Transplantation ◽  
Tyrosine Kinase ◽  
Cell Transplantation ◽  
Rare Variants ◽  
De Novo ◽  
Lymphoblastic Leukemia ◽  
Kinase Gene ◽  
Current Case

PCM1-JAK2 fusion mutations are rare variants that activate a tyrosine kinase leading to a variety of neoplasms that can involve any hematologic cell line. They most often present as myelodysplasia (MPD) and can demonstrate prominent eosinophilia and/or erythrodysplasia. Transformation to acute leukemia is often seen, as is de novo leukemia. Lymphomas have also been reported. The diagnosis can often be made with routine cytogenetic analysis but specific probes and detailed next generation sequencing may be necessary. JAK2 inhibitors are active in MPD as is stem cell transplantation. Transplantation has occasionally been successful in leukemic phase as well. The current case highlights both the difficulties in diagnosis as well as the second successful treatment of MPD, transformed into acute lymphoblastic leukemia.

Prolonged Survival in Philadelphia Positive Acute Lymphoblastic Leukemia Treated with Autologus Stem Cell Transplantation in the Inhibitor Tyrosine Kinase Era : A Retrospective Study

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5501-5501
Author(s):  
Camille Favennec ◽  
Marie-Lorraine Chretien ◽  
Caroline Legouge ◽  
Jean-Noel M. Bastie ◽  
Denis Caillot
Keyword(s):  
Stem Cell ◽  
Acute Lymphoblastic Leukemia ◽  
Stem Cell Transplantation ◽  
Tyrosine Kinase ◽  
Cell Transplantation ◽  
Lymphoblastic Leukemia ◽  
Prolonged Survival ◽  
White Blood Count ◽  
Allogenic Stem Cell Transplantation ◽  
Number Of Patients

Abstract With tyrosine kinase inhibitors (TKI) containing regimen and without allogenic stem cell transplantation (SCT), some studies showed prolonged survival in Philadelphia positive (Ph+) acute lymphoblastic leukemia (ALL), questioning the place of the allogenic SCT in the TKI era. We retrospectively reported the outcome of 39 patients with ALL Ph+ cured at the Bocage hospital, Dijon, France. The complete remission rate (CR) after induction was 100%, 1 patient died during consolidations. Two patients received an allogenic SCT and died of treatment related toxicity. Thirty six patients were referred to an autologus SCT strategy, in case of collection failure (7 patients) they received prolonged chemotherapy, for all TKI was given until progression or death. In this group the 5 years probability of overall survival and progression free survival are 60% and 53% respectively. Twenty two patients are alive in CR, 13 relapsed, 1 died in CR. Older age and high white blood count at diagnosis are bad prognostic factors. There were no correlation between minimal residual disease and prognosis. Our study showed prolonged survival is possible without allogenic SCT, in the TKI era. Autologus SCT is easily accessible and is associated with a very low mortality. These results should be confirmed by prospective studies including a larger number of patients. Disclosures No relevant conflicts of interest to declare.

Myeloablative allogeneic versus autologous stem cell transplantation in adult patients with acute lymphoblastic leukemia in first remission: a prospective sibling donor versus no-donor comparison

Blood ◽  
2009 ◽  
Vol 113 (6) ◽  
pp. 1375-1382 ◽  
Author(s):  
Jan J. Cornelissen ◽  
Bronno van der Holt ◽  
Gregor E. G. Verhoef ◽  
Mars B. van 't Veer ◽  
Marinus H. J. van Oers ◽  
...  
Keyword(s):  
Stem Cell ◽  
Acute Lymphoblastic Leukemia ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Lymphoblastic Leukemia ◽  
Adult Patients ◽  
Donor Group ◽  
No Donor ◽  
Sibling Donor ◽  
Standard Risk

Abstract While commonly accepted in poor-risk acute lymphoblastic leukemia (ALL), the role of allogeneic hematopoietic stem cell transplantation (allo-SCT) is still disputed in adult patients with standard-risk ALL. We evaluated outcome of patients with ALL in first complete remission (CR1), according to a sibling donor versus no-donor comparison. Eligible patients (433) were entered in 2 consecutive, prospective studies, of whom 288 (67%) were younger than 55 years, in CR1, and eligible to receive consolidation by either an autologous SCT or an allo-SCT. Allo-SCT was performed in 91 of 96 patients with a compatible sibling donor. Cumulative incidences of relapse at 5 years were, respectively, 24 and 55% for patients with a donor versus those without a donor (hazard ratio [HR], 0.37; 0.23-0.60; P < .001). Nonrelapse mortality estimated 16% (± 4) at 5 years after allo-SCT. As a result, disease-free survival (DFS) at 5 years was significantly better in the donor group: 60 versus 42% in the no-donor group (HR: 0.60; 0.41-0.89; P = .01). After risk-group analysis, improved outcome was more pronounced in standard-risk patients with a donor, who experienced an overall survival of 69% at 5 years (P = .05). In conclusion, standard-risk ALL patients with a sibling donor may show favorable survival following SCT, due to both a strong reduction of relapse and a modest nonrelapse mortality. This trial is registered with http://www.trialregister.nl under trial ID NTR228.

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