PMC4396885_01
Clinical course of a patient with RARS-T evolved to myelofibrosis. Red blood cell transfusion dependence (RBC TD) started in 2010 and ended in August 2012, with number of RBC units (U) per 4 weeks (wk) indicated by the arrows. Hemoglobin (Hb) levels are pretransfusion measurements during the period of RBC TD and are otherwise regular measurements. Hydroxyurea (HU) and/or anagrelide (AN) were given from February 2010 to June 2011 with multiple dose adjustments to keep the platelet count less than 1000 x 109/L. These were stopped as a requirement of a clinical trial of pomalidomide versus placebo (study medication indicated by SM); SM was given from July 2011 to January 2012 and stopped for lack of response. HU was resumed at doses of 2, 1.5, and 1 g/day, respectively, as indicated by the arrows (left to right), to keep the platelet count less than 1000, 400, and 400 x 109/L, respectively. Erythropoietin (EPO) was started in May 2012 at 40,000 U/wk and stopped in January 2013 because of a Hb of 130 g/dL. Deferasirox (DFX) was started in June 2012 (5 weeks after EPO) at a dose of 1500 mg/day (20 mg/kg/day). The patient's last RBC transfusion was on August 7, 2013.
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fig1
The Hb, SF levels, transfusion requirements, and medications received over the patient's course are shown in Figure 1.