SAF y leucemia promielocítica aguda: un desafío terapéutico

Abstract

cute promyelocitic leukemia (APL) is a subtype of leukemia which is associated with unique and distinctive coagulopathy. In the absence of treatment, it is rapidly fatal and even after initiation of therapy the major cause of early mortality is related to hemorrhagic complications. Antiphospholipid syndrome (APS) is a multisystem autoimmune disease associated with recurrent arterial and venous thrombosis and pregnancy loss. Recurrent thrombosis is the leading cause of mortality and long term anticoagulation therapy is required.  Patients with antiphospholipid antibodies have an increased risk of developing hematological neoplasms and the risk of thrombosis. We report a case of APL who developed after years of diagnosis of thrombotic APS.

A 51 years old man was referred for hematology and rheumatology evaluation to our hospital. He had a medical history of primary APS diagnosed 20 years ago. He had 2 episodes of Myocardial infarction (when he was 30 and 40 years old respectively) with triple positivity of antiphospholipid antibodies (LA, aCL Ig G and B2GPI Ig G) and he has been treated with oral anticoagulation with warfarin plus aspirin with INR of 3, statins and hydroxycloroquine. He developed dyspnea and tiredness. Laboratory findings showed pancytopenia: Hot 32.1%, HB 8.4 grs%, WBC 1400, platelets 20.000.  Antinuclear, ds-DNA, Sm, RNP, Ro and la antibodies were negative. Hepatitis B, C and syphilis serology was negative and complement levels were normal. CRP Sars-Cov2 was negative. Iron and folic acid levels were normal. BMP: 79% promyelocytes infiltration. Flow cytometry; 85% abnormal cells with immunophenotype of APL (CD34 -, HLA Dr -, CD117+, CD33 ++ (homogenous), CD13 ++ (heterogeneous), CD15 +/-, CD123 +, CD56 partial expression.). PML RAR ALFA (T 15,17):  presence of PML RAR ALFA band. He started treatment with ATRA and he completed induction and seven cycles of consolidation therapy. He required platelets transfusions and Low weight molecular heparin bridge therapy for short periods. He had no hemorrhage neither thrombotic complications. 50 days after BM examination showed remission.

The association of APL and APS is extremely rare and a diagnostic and therapeutic challenge. The onset of cytopenia in APS require a clinical and laboratory workup and bone marrow (BM) examination to determine the cause and for appropriate patient management. Common causes should be exclude including development of SLE, infection, iron and folic deficiency or effect of medications.