Hamostaseologie 2021; 41(02): 120-126
DOI: 10.1055/a-1393-8302
Review Article

Management of Disseminated Intravascular Coagulation in Acute Leukemias

Hugo ten Cate
1   Department of Internal Medicine and Thrombosis Expert Center, Maastricht University Medical Center, Maastricht, The Netherlands
2   CARIM School for Cardiovascular Diseases, Maastricht, The Netherlands
Avi Leader
2   CARIM School for Cardiovascular Diseases, Maastricht, The Netherlands
3   Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
4   Hematology Institute, Rabin Medical Center, Petah Tikva, Israel
› Author Affiliations


Disseminated intravascular coagulation (DIC) is characterized by the intravascular activation of coagulation with loss of localization arising from different causes, and is diagnosed using scoring systems which rely upon the presence of an underlying disorder compatible with DIC alongside hemostatic derangements such as low platelet count, prolonged prothrombin time, and elevated fibrinogen degradation products. DIC is common in patients with acute leukemia, with prevalence ranging from 17 to 100% in acute promyelocytic leukemia (APL) and 8.5 to 25% in acute lymphoblastic leukemia (ALL) and non-APL acute myeloid leukemia (AML). The pathophysiology is complex and varies between the leukemia subtypes, and is not fully reflected by the laboratory markers currently used to classify DIC. Similarly, the clinical consequence of DIC in acute leukemia also varies across the types of leukemia. DIC is primarily associated with bleeding in APL, while thrombosis is the dominant phenotype in ALL and non-APL AML. The cornerstone of managing DIC is the treatment of the underlying disease, as exemplified by the important role of early administration of all-trans retinoic acid in APL. Other aspects of management focus on supportive care aimed at minimizing the risk of bleeding, via transfusion of blood products. The use of blood products is more liberal in APL, due to the hemorrhagic phenotype and unacceptably high rates of early hemorrhagic death. This review will focus on the pathophysiology, risk factors, clinical implications, and the management of DIC in patients across the spectrum of acute leukemias.

Publication History

Received: 10 December 2020

Accepted: 15 February 2021

Article published online:
15 April 2021

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