Paravertebral extramedullary haemopoiesis occurring in a case of congenital dyserythropoietic anaemia type II

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Paravertebral extramedullary haemopoiesis occurring in a case of congenital dyserythropoietic anaemia type II

A 63-year-old man, previously diagnosed with hereditary spherocytosis and hereditary haemochromatosis, was referred with a raised ferritin of 1238 lg/l. Haemoglobin and C-reactive protein were normal; a fasting transferrin saturation of 95% confirmed iron overload and HFE mutation studies revealed C282Y heterozygosity. Liver biopsy showed grade IV haemosiderosis and established cirrhosis. The patient was commenced on a venesection programme. At routine follow-up, the patient produced a short history of anorexia, weight loss and abdominal pain. Haemoglobin was 115 g/l with an acute phase response. Peripheral blood morphology showed anisocytosis, occasional nucleated red cells and postsplenectomy changes. A computed tomography scan (top left) revealed paravertebral masses with small volume abdominal lymphadenopathy. Needle biopsy of a paravertebral mass stained with anti-glycophorin-C confirmed extramedullary haemopoiesis (top right). Biopsy of retroperitoneal nodes showed features of an inflammatory, reactive process. On bone marrow aspirate (bottom left) and trephine biopsy (bottom right) there was a predominance of binucleated normoblasts and storage iron was increased. Ham’s acidified serum test was positive with donor serum and patient red cells had elevated i

expression, confirming the true diagnosis of congenital dyserythropoietic anaemia type II (HEMPAS) with secondary iron overload. The patient’s clinical condition improved following the discontinuation of venesections. On repeat scanning, the lymphadenopathy had reduced in size and remains under observation. Paravertebral masses consisting of extramedullary haemopoietic tissue are most frequently encountered in cases of thalassaemia intermedia where the stimulus to haemopoiesis is not suppressed by transfusion. It is postulated that in a patient with ineffective erythropoiesis, venesection stimulated significant extramedullary haemopoiesis and was responsible for the patient’s clinical deterioration. Agha Imran1 Rod Mawhinney1 David Swirsky2 Claire Hall1 1

Department of Haematology, Harrogate District Hospital, Harrogate,


HMDS, Leeds General Infirmary, Leeds, UK. E-mail: [email protected]

ª 2007 The Authors Journal Compilation ª 2007 Blackwell Publishing Ltd, British Journal of Haematology, 140, 1

First published online 14 September 2007 doi:10.1111/j.1365-2141.2007.06808.x

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