Ruxolitinib withdrawal

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Ruxolitinib withdrawal Various toxicities secondary to ruxolitinib discontinuation syndrome: case report

A 70-year-old woman developed ruxolitinib discontinuation syndrome (RDS) presenting as massive splenomegaly, perisplenic haemorrhage, and colitis, decreased platelet counts and hypoxic respiratory failure following discontinuation of ruxolitinib treatment for polycythaemia vera. The woman was diagnosed with primary polycythemia vera in June 2012 and started receiving ruxolitinib [route and dosage not stated] in in October 2012. However, she progressed to myelofibrosis in 2017 with resulting splenomegaly. In September 2018, she was found to have elevated leukocyte count to 90,000/mL with 84% blasts, which signified transformation to acute myeloid leukaemia. Therefore, she was hospitalised for the treatment of hyper-leukocytosis. Her ruxolitinib was withheld, and she was treated with leukapheresis. Subsequent, bone marrow biopsy confirmed acute myeloid leukaemia. On day 3 of admission, she acutely developed left-upper-quadrant pain. An abdominal CT scan revealed worsened massive splenomegaly, perisplenic haemorrhage, and colitis. CT angiogram demonstrated new inflammatory ground-glass opacities. Hypoxic respiratory failure developed subsequently. Laboratory investigations ruled out infection and pulmonary embolism. Hence, diagnosis of RDS was suspected which was also supported by decreased platelet count. She was treated with prednisone daily with minimal effect. The woman resumed ruxolitinib later. Subsequently, a day after resuming ruxolitinib, her abdominal pain and respiratory distress significantly improved. She was eventually discharged on prednisone and ruxolitinib taper. She remained asymptomatic, with splenomegaly back at baseline. Her cell counts also recovered, allowing initiation of azacitidine treatment for acute myeloid leukaemia. Shakir AT, et al. Ruxolitinib discontinuation syndrome in a patient with myelofibrosis to acute myeloid leukemia transformation. JCO Oncology Practice 16: 395-396, No. 7, 803501014 Jul 2020. Available from: URL: http://doi.org/10.1200/JOP.19.00179

» Editorial comment: Details of this case report have previously been published [see Reactions 1787 p446; 803447619]

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Reactions 12 Sep 2020 No. 1821