Acute promyelocytic leukemia natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [3]; Grammar Reviewer: Natalie Harpenau, B.S.[4]

Overview

The natural history of acute promyelocytic leukemia is characterized by symptoms related to defective normal blood cell production. These symptoms include fatigue, bleeding, and infection. Complications include thrombosis and hemorrhage, which eventually occur in a significant proportion of patients. Early death is common and is related to bleeding complications. Therapy-related complications include differentiation syndrome, QT interval prolongation, and cardiomyopathy. The prognosis of acute promyelocytic leukemia was previously poor, but the advent of arsenic trioxide and all-trans retinoic acid has rendered the prognosis to be far more favorable in the recent years.

Natural History

Complications

In a 2015 study from MD Anderson Cancer Center, it was shown that the annual incidence of venous thromboembolism, which includes deep vein thrombosis and pulmonary embolism, was 6.1-42%, which is the highest amongst all leukemia sub-types. In contrast, the incidence of venous thromboembolism in chronic myeloid leukemia was only 1.5%.[7]

Disease Thrombotic Incidence

Acute promyelocytic leukemia

6.1-48%

Acute myeloid leukemia

3.7%

Chronic lymphocytic leukemia

2.7%

Acute lymphoblastic leukemia

2.1-13%

Chronic myeloid leukemia

1.5%

Prognosis

  • Prior to the introduction of readily available diagnostics and targeted therapeutics, the prognosis of acute promyelocytic leukemia was previously very poor, especially in the early phase of the disease.[31][32]
  • The poor prognosis was due to high bleeding risk and death from hemorrhagic complications due to disseminated intravascular coagulation. Death typically occurs within a few days to weeks in the absence of treatment. The early death rate is estimated to be 17.3%, based on a large population-based analysis that was conducted in the United Stated between 1992-2007.[33] [2]
  • The 5-year survival rate is only 30-40% after 5 years in younger patients.[23] In the current era of medicine (after the introduction of all-trans retinoic acid and arsenic trioxide), the prognosis of acute promyelocytic leukemia carries a much better prognosis.[3]
  • Patients can achieve long-term, durable remission if treated appropriately in an expedited manner with medications such as all-''trans'' retinoic acid, arsenic trioxide, or cytotoxic chemotherapy. The current overall survival rate is 86-97%, and the complete remission rate is 90-100%.[23]
  • In a multi-center study published in 2017 that evaluated the long-term outcomes of patients with acute promyelocytic leukemia, the complete remission rate was 96% [34] and induction mortality if low at 4%.[34]

References

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  4. 4.0 4.1 Choudhry, Aditi; DeLoughery, Thomas G. (2012). "Bleeding and thrombosis in acute promyelocytic leukemia". American Journal of Hematology. 87 (6): 596–603. doi:10.1002/ajh.23158. ISSN 0361-8609.
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  11. . doi:10.1182/blood-2016-09-739334 PMCID: PMC5374289 Check |doi= value (help). Missing or empty |title= (help)
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  32. Efficace, Fabio; Breccia, Massimo; Avvisati, Giuseppe; Cottone, Francesco; Intermesoli, Tamara; Borlenghi, Erika; Carluccio, Paola; Rodeghiero, Francesco; Fabbiano, Francesco; Luppi, Mario; Romani, Claudio; Sborgia, Marco; D’Ardia, Stefano; Nobile, Francesco; Cantore, Nicola; Crugnola, Monica; Nadali, Gianpaolo; Vignetti, Marco; Amadori, Sergio; Lo Coco, Francesco (2018). "Health-related quality of life, symptom burden, and comorbidity in long-term survivors of acute promyelocytic leukemia". Leukemia. doi:10.1038/s41375-018-0325-4. ISSN 0887-6924.
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