Abstract
BackgroundAlthough life-threatening hemorrhage is a usual manifestation of acute promyelocytic leukemia (APL), thrombotic events seem to be more common in APL compared to other subtypes of acute leukemia. The treatment and prophylaxis of thrombosis are controversial due to the high risk of bleeding caused by disseminated intravascular coagulation (DIC) and thrombocytopenia. To the best of our knowledge we report the first case of APL in a patient with prosthetic heart valves successfully treated with a combination of all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO). We hope this case report helps clinicians to manage different spectra of coagulopathy in APL successfully.Case presentationA 38-year-old Asian man presented with diagnosis of APL confirmed by bone marrow biopsy. He was on warfarin due to prosthetic mitral and aortic valves. He was at risk of both hemorrhagic events due to DIC and life-threatening valve thrombosis. Our management regimen included unfractionated heparin adjusted according to the platelet count to prevent both valve thrombosis and bleeding events. The patient tolerated treatment well without any hemorrhagic or thrombotic events, and complete molecular remission was achieved by ATRA and ATO without the need for chemotherapeutic agents.ConclusionAlthough this case is exceptional, a precise evaluation may be needed to select the appropriate dose and type of anticoagulant to treat a patient with APL.
Highlights
Acute promyelocytic leukemia (APL) is a specified subtype of acute myelogenous leukemia (AML) characterized by thrombo-hemorrhagic syndrome [1]
acute promyelocytic leukemia (APL) is associated with the presence of hallmark cytogenetic abnormality t(15;17) which results from the fusion of the retinoic acid alpha (RARalpha) and PML genes [3]
We considered the diagnosis of low-risk APL and immediately started the patient on all-trans-retinoic acid (ATRA) at 45 mg/m2 and arsenic trioxide (ATO) at the standard dose of 0.15 mg/kg
Summary
Acute promyelocytic leukemia (APL) is a specified subtype of acute myelogenous leukemia (AML) characterized by thrombo-hemorrhagic syndrome [1]. We report a 37-year-old man with prosthetic mitral and aortic valves who presented with APL and coagulopathy. The results of other aspects of the physical examination were unremarkable except for a loud opening click related to the prosthetic valves He had undergone prosthetic mitral and aortic valve replacement 16 years prior to the current presentation, for which he was on the oral anticoagulant warfarin. The disseminated intravascular coagulation (DIC) profile showed an increase in PT and D-dimer and a decrease in fibrinogen level, the patient did not receive fresh frozen plasma or cryoprecipitate due to lack of any evidence of active bleeding and the possible risk of valve thrombosis. Following recovery of the peripheral blood cell count on day 20 of therapy with ATRA and ATO, heparin was changed to warfarin and the patient was discharged from hospital. The patient is currently in molecular complete remission at 16 months from diagnosis
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