Management of Disseminated Intravascular Coagulation in Acute Leukemias

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.

Chest Pain Diagnosed as Acute Leukemia: Focus on Coagulation Abnormalities Rather Than White Blood Cell Count

“…When leukocytes in the peripheral blood are normal, coagulopathy may be a clue to the diagnosis of AL. Acute promyelocytic leukemia (APL) has a high frequency of coagulopathy, with a DIC complication rate of 78.0% compared to 31.6% for AML excluding APL [ 12 ]. Although this case was diagnosed as AML cuplike, a report of the same type of cases showed a DIC complication rate of 57.1%, suggesting that this type of patient may be prone to DIC as well as APL [ 13 ].…”

Budd-Chiari Syndrome as an Initial Presentation of Non-Promyelocytic Acute Myelogenous Leukemia

“…Although we did not initially do so, we recommend considering checking D-dimer levels in the evaluation of newly diagnosed AML patients. DIC commonly occurs in the setting of BCS due to APL with prevalence ranging from 17% to 100% [ 7 , 11 , 21 ]. DIC is mainly associated with bleeding in APL, while thrombosis is common in acute lymphocytic leukemia (ALL) and non-APL AML [ 21 ].…”

“…DIC commonly occurs in the setting of BCS due to APL with prevalence ranging from 17% to 100% [ 7 , 11 , 21 ]. DIC is mainly associated with bleeding in APL, while thrombosis is common in acute lymphocytic leukemia (ALL) and non-APL AML [ 21 ]. In the study reported by Amitrano et al [ 7 ] of BCS due to non-promyelocytic AML, no DIC was reported; however, in our case, DIC was suspected due to the patient having both clinical (hepatic vein thrombosis) and laboratory findings consistent with DIC.…”

Disseminated Intravascular Coagulation: The Past, Present, and Future Considerations

“…Acute leukemia is frequently complicated with DIC, with up to 100% prevalence in acute promyelocytic leukemia (APL) and between 8.5 and 25% in acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). 76 In APL, high levels of annexin II on leukemic cells are responsible for the upregulation of the fibrinolytic system. 77 Annexin II has a high affinity for both plasminogen and its activator tissue-type plasminogen activator (t-PA), and facilitates plasminogen activation leading to increased fibrinolysis.…”