What's new in the pathogenesis of the coagulopathy in acute promyelocytic leukemia?

Simon Mantha*, Martin S. Tallman, Gerald A. Soff

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

45 Scopus citations


Purpose of review Acute promyelocytic leukemia (APL) is associated with a complex coagulopathy. In spite of substantial recent improvements in treatment regimens, hemorrhagic death remains the main cause of induction failure. In this review, we delineate recent understanding of the pathophysiology and management of the hemorrhagic diathesis of APL. Recent findings Laboratory and clinical data suggest that the malignant leukocytes mediate the hemorrhagic diathesis associated with APL through multiple mechanisms which lead to a combination of consumptive coagulopathy and primary hyperfibrinolysis. Exposure of tissue factor and Annexin II by the leukemic blasts is the main determinants of these processes. Promyelocyte-derived microparticles have recently been implicated in the coagulopathy as well. Total white cell count and platelet count have emerged as good general predictors of hemorrhagic death, along with the different routine hemostatic parameters. Prompt treatment with all-trans retinoic acid, with or without arsenic trioxide, is the most important step in preventing bleeding complications. Repletion of coagulation factors and platelets with blood products remains the mainstay of supportive treatment, whereas the role of recombinant soluble thrombomodulin is currently being investigated. Summary The coagulopathy of APL is multifactorial, with both disseminated intravascular coagulation and primary hyperfibrinolysis mediated largely by the malignant leukocytes.

Original languageEnglish (US)
Pages (from-to)121-126
Number of pages6
JournalCurrent opinion in hematology
Issue number2
StatePublished - Mar 1 2016


  • acute promyelocytic leukemia
  • disseminated intravascular coagulation
  • hemorrhage
  • hyperfibrinolysis

ASJC Scopus subject areas

  • Hematology


Dive into the research topics of 'What's new in the pathogenesis of the coagulopathy in acute promyelocytic leukemia?'. Together they form a unique fingerprint.

Cite this