Favorable Outcome of Budd-Chiari Syndrome in Acute Promyelocytic Leukemia

Acute promyelocytic leukemia (APL) is characterized by severe haemorrhagic diathesis as the major cause of treatment failure and fatal outcome. Less frequently the patient may either present with thrombosis or it may complicate remission induction therapy. We present a 49-year-old female with a low-risk APL, with translocation t(15;17), bcr3 isoform transcript, and an aberrant immunophenotype expression of CD2 and CD56 antigens. She developed thrombosis of the left hepatic vein during remission induction with idarubicin and all-trans-retinoic acid(ATRA). She received prophylaxis with low molecular weight heparin (LMWH). After four months of anticoagulant therapy, Doppler ultrasonography showed a complete recanalisation of the left hepatic vein. Besides APL, she had a thrombophylia, methylentetrahydrofolate reductase (C677T) gene mutation which contributed as an extra risk factor towards thrombosis. The patient achieved complete cytologic, cytogenetic and molecular remission after completing antileukemic treatment combined with a LMWH.
In patients diagnosed with APL and concomitant thrombotic events, the screening test for inherited and/or acquired thrombophilia are highly recommended.