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The 2023 JSH practical guidelines for hematological malignancies: ALL
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The 2023 JSH practical guidelines for hematological malignancies provide a treatment algorithm and recommendations for patients with ALL in Japan.1 These recommendations were published in the International Journal of Hematology by Hatta et al.1
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| Key learnings |
| For patients with Ph+ ALL, TKI combined with chemotherapy is recommended, with allo-HSCT in first remission. In patients aged ≥65 years, TKI plus a steroid is recommended, with RIC and maintenance chemotherapy if possible. Preemptive TKI therapy is advised if MRD is detected post allo-HSCT; however, prophylactic TKI maintenance is not recommended if the patient is MRD-negative. TKI maintenance therapy for ≥5 years is recommended for patients not undergoing allo-HSCT in first remission. |
| For patients with Ph− ALL, pediatric-type chemotherapy is recommended for adolescents and young adults. For patients aged 40–64 years, high-dose methotrexate is recommended. Patients treated with pediatric-type protocols in first remission should continue chemotherapy, but allo-HSCT should be considered in patients with unfavorable prognostic factors. Standard therapy for patients aged ≥65 years is under development and current treatment options include combination chemotherapy or palliative steroid therapy tailored to individual patient needs. |
| MRD assessment after induction therapy is crucial. The optimal timing of subsequent MRD assessment varies depending on the treatment. Patients with MRD ≥0.01% after induction are at high risk of relapse and MRD monitoring should guide further treatment, including the timing of allo-HSCT. |
| For patients in remission, CNS prophylaxis with intrathecal anticancer drugs and either high-dose methotrexate or high-dose cytarabine is recommended for all adult patients. CNS prophylaxis with cranial irradiation is acceptable in high-risk patients. |
| For patients with R/R ALL undergoing reinduction therapy, prior treatments should be considered. Blinatumomab and inotuzumab ozogamicin are recommended for patients with CD19-positive and CD22-positive B-ALL, respectively. Later-generation TKIs can be considered in patients with Ph+ ALL who relapsed after earlier-generation TKIs. Nelarabine is a treatment option for patients with T-ALL. CAR T-cell therapy is an option for patients aged <25 years with CD19-positive B-ALL who did not achieve a second remission or relapsed after allo-HSCT. |
Abbreviations: ALL, acute lymphoblastic leukemia; allo-HSCT, allogeneic hematopoietic stem cell transplantation; B-ALL, B-cell ALL; CAR, chimeric antigen receptor; CNS, central nervous system; JSH, Japanese Society of Hematology; MRD, measurable residual disease; Ph, Philadelphia chromosome; R/R, relapsed/refractory; RIC, reduced-intensity conditioning; T-ALL, T-cell ALL; TKI, tyrosine kinase inhibitor.
- Hatta Y, Izutsu K, Onizuka M, et al. JSH practical guidelines for hematological malignancies, 2023: leukemia-3. Acute lymphoblastic leukemia/lymphoblastic lymphoma: ALL/LBL. Int J Hematol. 2024;120(4):394–413. DOI: 1007/s12185-024-03836-7
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