LAUSR

A 45-year-old woman with sickle cell/ß thalassemia due to the HBB :c.92 + 5G>C severe ß + thalassemia mutation, who had been on a clinical trial of voxelotor for 4 years was assessed clinically and hematologically during follow-up. Concomitantly she was taking hydroxycarbamide (hydroxyurea) at a stable dosage. During this period of combined therapy, her hemoglobin concentration (Hb) had risen from 86 to 103 g/L and mean cell hemoglobin concentration (MCHC) from 327 to 346 g/L with a varying mean cell volume (MCV) of 79 – 90 fL. Her blood film having been typical of sickle cell disease pre-voxelotor, now showed only rare sickle cells and small numbers of boat-shaped or spindle-shaped cells, some of which appeared dense (images above, modified Wright – Giemsa stain (cid:1) 100 objective). In addition, there were hyposplenic fea-tures (Howell – Jolly bodies, target cells, Pappenheimer bodies, acanthocytes, and large platelets) together with nucleated red blood cells and macrocytes, some of which were polychromatic.