Acute promyelocytic leukemia developed in a patient with congenital antithrombin III deficiency

A case of acute promyelocytic leukemia (APL) developed in a patient with congenital antithrombin III (AT-III) deficiency is reported. Despite the presence of disseminated intravascular coagulation (DIC), plasma AT-III activity was not decreased at the diagnosis of APL compared to the patient's baseline level (approximately 50% of normal). He was successfully treated with all-trans retinoic acid (ATRA) to achieve complete remission without the use of heparin. Although he developed phlebitis at the site of insertion of the intravenous catheter during remission-induction, no major thrombotic episode was noted. Coagulation parameters including fibrin and fibrinogen degradation products (FDP-E), thrombin-antithrombin complex (TAT), FDP-D dimer (D-D dimer), and plasmin-alpha 2 plasmin inhibitor complex (PIC) improved rapidly after initiation of ATRA. This case is a clear demonstration of the characteristics of DIC developing in APL, i.e. no or minimal decrease in the level of AT-III activity and a predominant increase in the fibrinolytic system, rather than hypercoagulability.