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The 2023 JSH practical guidelines for hematological malignancies: ALL

By Dylan Barrett

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Oct 22, 2024

Learning objective: After reading this article, learners will be able to cite a new clinical development in acute lymphoblastic leukemia.


The 2023 JSH practical guidelines for hematological malignancies provide a treatment algorithm and recommendations for patients with ALL in Japan.These recommendations were published in the International Journal of Hematology by Hatta et al.1

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. 

References

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