Enhanced Survival in Acute Promyelocytic Leukemia

Enhanced Survival in Acute Promyelocytic Leukemia: A New Era of Therapeutic and Supportive Interventions

Muhammed Usman Shaikh1

  1. Department of Oncology, Aga Khan University Hospital, Karachi-Pakistan

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PUBLISHED: 31 January 2025

CITATION: SHAIKH, Muhammed Usman; SAEED, Nabiha. Enhanced Survival in Acute Promyelocytic leukemia: A New Era of Therapeutic and Supportive Interventions. Medical Research Archives,  Available at: <https://esmed.org/MRA/mra/article/view/6119>.

COPYRIGHT: © 2025 European Society of Medicine. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

DOI: https://doi.org/10.18103/mra.v13i1.6119

 ISSN 2375- 1924

Abstract

Acute promyelocytic leukemia is a relatively uncommon type of acute myeloid leukemia, accounting for 5-8% of acute myeloid leukemia cases. Patients are usually younger, with a median age at diagnosis of 40 years. The diagnostic parameters used to confirm the diagnosis of acute promyelocytic leukemia include the characteristic morphology of leukemic cells and typical flow cytometry findings, such as CD34 and HLA-DR negativity. Acute promyelocytic leukemia was previously classified as AML-M3 in the older French-American-British (FAB) classification system; and with the advancement in cytogenetic and genetic research, the prognosis of acute promyelocytic leukemia has improved significantly. However, the incidence of early induction mortality remains high, with rates ranging from 82% to 36%. From recent studies, the incorporation of arsenic trioxide and all-trans retinoic acid in patients with acute promyelocytic leukemia has shown significant improvements in overall survival and reduced induction mortality. This review aims to highlight the recent advancements in the management of acute promyelocytic leukemia and the role of therapeutic and supportive interventions.

Keywords

  • Acute promyelocytic leukemia
  • Therapeutic interventions
  • Supportive interventions
  • All-trans retinoic acid
  • Arsenic trioxide

Introduction

Acute promyelocytic leukemia is a relatively uncommon type of acute myeloid leukemia, accounting for 5-8% of acute myeloid leukemia cases. Patients are usually younger, with a median age at diagnosis of 40 years. The diagnostic parameters used to confirm the diagnosis of acute promyelocytic leukemia include the characteristic morphology of leukemic cells and typical flow cytometry findings, such as CD34 and HLA-DR negativity. Acute promyelocytic leukemia was previously classified as AML-M3 in the older French-American-British (FAB) classification system; and with the advancement in cytogenetic and genetic research, the prognosis of acute promyelocytic leukemia has improved significantly. However, the incidence of early induction mortality remains high, with rates ranging from 82% to 36%. From recent studies, the incorporation of arsenic trioxide and all-trans retinoic acid in patients with acute promyelocytic leukemia has shown significant improvements in overall survival and reduced induction mortality. This review aims to highlight the recent advancements in the management of acute promyelocytic leukemia and the role of therapeutic and supportive interventions.

Figure 1: Diagnostic parameters used to confirm acute promyelocytic leukemia.
Figure 1: Diagnostic parameters used to confirm acute promyelocytic leukemia.

Management of Coagulopathy

The management of coagulopathy has improved over time with the prophylactic administration of blood and blood products. The European LeukemiaNet and other regional guidelines provide sufficient evidence to prevent coagulopathy, including maintaining platelets at ≥300,000/µL, fibrinogen at ≥150 mg/dL, and fresh frozen plasma for deranged prothrombin time, activated partial thromboplastin time, and other coagulation factors.

Figure 2: Management of coagulopathy in acute promyelocytic leukemia.
Figure 2: Management of coagulopathy in acute promyelocytic leukemia.

Induction Mortality

Despite all the advancements in the management of acute promyelocytic leukemia, early deaths or induction mortality (usually defined as death within 30 days of starting targeted therapy) remain a concern for treating physicians. Once diagnosed, it is crucial to start all-trans retinoic acid as soon as possible, along with the prophylactic and therapeutic use of blood products.

Induction Deaths in Acute Promyelocytic Leukemia: Data from India and Pakistan
Study Number of patients Deaths Mortality %
India 2021 Rajani et al. 111 21 19%
India 2021 Tejasvi et al. 64 9 14%
India 2021 Bajpai et al. 33 8 24%
India 2024 K Sindhu 40 23 58%
Pakistan 2024 Naz et al. 50 17 32%
Pakistan 2020 Shaikh Raza et al. 30 12 30%

Conclusion

In conclusion, the management of acute promyelocytic leukemia is a complex process involving preventive infusion of blood products and corticosteroids. Despite this, induction mortality remains a major challenge, but there are improvements with early induction and prophylactic administration of blood and blood products.

References

1. Sanzi MA, McKenna RL, Ellinger G, Brunning RD. Classification of acute promyelocytic leukemia by FAB criteria: Chronic and acute leukemias in adults: Springer, 1975: 147-74.

2. Bennett JM, Catovsky D, Daniel MT, Flincking G, Galton DA, Gralnick HR, et al. Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet. Blood. 2009;113(19):1875-91.

3. Taraboletti G, Borstotti P, Chirivi RG, Vergani V, Falinga A, Barbui T, et al. Effect of all-trans retinoic acid (ATRA) on the adhesive and motility properties of acute promyelocytic leukemia cells. International journal of cancer. 1997;70(1):72-7.

4. Vaid T, Aggarwal M, Kumar P, Dhawan R, Das J, Viswanathan G, et al. Clinical Profile, Complications and Outcomes of Patients with Acute Promyelocytic Leukemia: Indian Perspective. Blood. 2021;138:4385.

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