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Plain film signs of sickle β-thalassaemia

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A 28-year-old woman of Jewish descent with a history of sickle β-thalassaemia disorder and uterine fi broids presented to the emergency department with a 2-week history of frank haematuria. She… Click to show full abstract

A 28-year-old woman of Jewish descent with a history of sickle β-thalassaemia disorder and uterine fi broids presented to the emergency department with a 2-week history of frank haematuria. She had been taking paracetamol daily for persistent headaches. On examination she was pale and tachycardic and had a palpable spleen. Full blood count showed haemoglobin 60 g/L. Creatinine was 97 μmol/L. Intravenous urogram showed pyelotubular refl ux, splenomegaly, gallstones, uterine fi broids, and coarsening of the pelvic bones and spine. We believe that the analgesic acted synergistically with the existing hypoxia of the renal medulla caused by her chronic anaemia state leading to medullary ischaemia and renal papillary necrosis, causing gross haematuria and the pyelotubular refl ux evident on radiography. The coarse appearance of bone seen on the plain fi lm is due to bone marrow expansion, and the mass eff ect on the left kidney caused by her splenomegaly; the lateral displacement of the ureters is likely due to uterine fi broids. Paracetamol was stopped and she was admitted by the nephrologists for intravenous hydration. She received two units of packed red cells and was discharged 3 days later after resolution of her haematuria, with haemoglobin 82 g/L.

Keywords: sickle thalassaemia; thalassaemia; film signs; uterine broids; plain film

Journal Title: The Lancet
Year Published: 2017

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