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Acute lymphoblastic leukemia

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Added 2023 ESMO, 2020 ISTH, and 2017 CAP/ASH guidelines for the diagnosis and management of acute lymphoblastic leukemia.

Background

Overview

Definition
ALL is a hematological malignancy that originates from B- or T-lymphoid progenitor cells and is characterized by the abnormal proliferation and accumulation of immature lymphoid cells within the bone marrow and lymphoid tissues.
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Pathophysiology
The pathophysiology of ALL is complex and involves a variety of genetic abnormalities that disrupt normal cell maturation and promote the uncontrolled proliferation of immature lymphoid cells.
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Epidemiology
The incidence of ALL is estimated at 1.8 per 100,000 person-years in the US and 2.75 per 100,000 person-years in Europe.
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Disease course
ALL typically presents with symptoms such as fever, pallor, bleeding, lymphadenopathy, and hepatosplenomegaly. Patients may also have lymphoblasts in the peripheral blood and bone marrow. In some cases, musculoskeletal manifestations may be the only initial presenting symptom.
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Prognosis and risk of recurrence
The prognosis is based on age at diagnosis, WBC count at presentation, and the presence of certain genetic changes. The outcome for older adults (≥ 40 years) and patients with relapsed or refractory ALL remains poor.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of acute lymphoblastic leukemia are prepared by our editorial team based on guidelines from the American Society for Transplantation and Cellular Therapy & Cell Therapy Transplant Canada (ASTCT 2024), the European Society of Medical Oncology (ESMO 2024,2016), the International Society on Thrombosis and Haemostasis (ISTH 2020), and the College of American ...
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Classification and risk stratification

Classification: as per ASH/CAP 2017 guidelines, use the current WHO terminology for the final diagnosis and classification of acute leukemia.
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  • Risk assessment

Diagnostic procedures

Biopsy and histopathology: as per ASH/CAP 2017 guidelines, perform fresh bone marrow aspiration in all patients with suspected acute leukemia, and use a portion of aspirate to make smears for morphologic evaluation. Evaluate adequate bone marrow trephine core biopsy, bone marrow trephine touch preparations, and/or marrow clots in conjunction with the bone marrow aspirates, if performed.
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  • Chromosomal and genetic testing

  • Lumbar puncture

Medical management

Management of newly diagnosed disease, induction and maintenance chemotherapy: as per ESMO 2016 guidelines, offer induction chemotherapy for 1-2 months, followed by consolidation cycles (alternating) for 6-8 months and maintenance therapy for 2-2.5 years.
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  • Management of newly diagnosed disease (targeted therapy, Ph-)

  • Management of newly diagnosed disease (targeted therapy, Ph+)

  • Management of newly diagnosed disease (targeted therapy, Ph-like)

  • Management of newly diagnosed disease (targeted therapy, immune checkpoint inhibitors)

  • Management of newly diagnosed disease (stem cell transplantation)

  • Management of relapsed or refractory disease

  • Management of L-asparaginase-related hypofibrinogenemia

  • Management of thrombocytopenia

  • Management of DIC

  • Management of donor-specific anti-HLA antibodies (prevention)

  • Management of donor-specific anti-HLA antibodies (testing)

  • Management of donor-specific anti-HLA antibodies (desensitization therapy)

  • Management of donor-specific anti-HLA antibodies (follow-up)

  • Management of non-donor-specific anti-HLA antibodies