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Management of Disseminated Intravascular Coagulation in Acute Leukemias
Hugo Ten Cate1,2, Avi Leader2,3,4
1Department of Internal Medicine and Thrombosis Expert Center, Maastricht University Medical Center, Maastricht, The Netherlands.
Insights
Disseminated intravascular coagulation (DIC) in acute leukemia presents differently across subtypes, often leading to bleeding in APL and thrombosis in ALL/AML. Effective management hinges on treating the underlying leukemia and providing supportive care.
Area of Science:
- Hematology
- Oncology
- Coagulation Disorders
Background:
- Disseminated intravascular coagulation (DIC) involves widespread coagulation activation and is diagnosed via scoring systems based on laboratory markers and underlying conditions.
- DIC is prevalent in acute leukemias, with varying rates in acute promyelocytic leukemia (APL), acute lymphoblastic leukemia (ALL), and non-APL acute myeloid leukemia (AML).
- Current diagnostic markers and understanding of DIC pathophysiology in acute leukemia are incomplete, showing complexity across subtypes.
Purpose of the Study:
- To review the pathophysiology, risk factors, clinical manifestations, and management strategies for DIC in acute leukemia patients.
- To highlight the differential impact of DIC, including bleeding versus thrombosis, across various acute leukemia subtypes.
- To emphasize the importance of treating the underlying leukemia as the primary management approach for DIC.
Main Methods:
- Literature review focusing on studies investigating DIC in acute leukemia.
- Analysis of existing data on DIC prevalence, pathophysiology, and clinical outcomes in APL, ALL, and AML.
- Synthesis of current management guidelines and treatment approaches for DIC in this patient population.
Main Results:
- DIC pathophysiology and clinical consequences differ significantly among acute leukemia subtypes.
- APL-associated DIC predominantly causes bleeding, necessitating liberal blood product transfusion.
- ALL and non-APL AML-associated DIC are more commonly linked to thrombosis.
Conclusions:
- DIC management in acute leukemia requires a subtype-specific approach, prioritizing treatment of the underlying malignancy.
- Early intervention with agents like all-trans retinoic acid is crucial for APL-related DIC.
- Supportive care, including judicious use of blood products, is essential for managing DIC complications in acute leukemia.
Abstract:
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.
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