LAUSR

A 62-year-old Turkish Cypriot man who was known to have hemoglobin H diseasewith the genotype– –/aa due to coinheritance of a thalassemia and a non-deletional mutation of the alpha2 globin gene, IVS-I donor site 5bp deletion (HBA2:c.9512_9516delTGAGG), that preventsmRNA splicing. He had been transfusion-independent since undergoing splenectomy 20 years earlier. Other past medical history included recurrent gout, pulmonary embolism, stage IV chronic kidney disease, chronic hepatitis B infection, and asthma. On routine tests his hemoglobin concentration had dropped to 67 g/L (from a baseline of around 90– 100 g/L) with a mean cell volume of 56 fL (baseline 67 fL). He had mild shortness of breath. This deterioration was found to be due to superimposed iron deficiency related to chronic gastro-intestinal bleeding from colonic angiodysplastic lesions found on colonoscopy. The blood film showed multiple abnormalities (top Images). There was severe microcytosis, hypochromia, striking poikilocytosis, and target cells. Post-splenectomy changes were also seen: acanthocytes, Howell– Jolly bodies, and giant platelets. There were red cell fragments, some of which were acanthocytic while others contained Howell–Jolly bodies or Pappenheimer bodies. Of particular interest, however were unusual inclusions that are specific for hemoglobin H disease post-splenectomy. These were round and dense with the staining characteristics of hemoglobin. They appeared to be bound to the red cell membrane. They resembled the