Renal myelofibrosis: an unusual cause of renal impairment.

previously described [3]. EMH may also involve periCase History renal tissue and the collecting system [4–6 ]. In this case extramedullary haematopoiesis was found in the A 58-year-old man, diagnosed with myelofibrosis 9 renal medulla and in the hilar connective tissue where years previously, presented with a 2 month history of it was associated with myelofibrotic tissue similar to increasing breathlessness, malaise and ankle oedema. that found in the marrow and the lymph node. It is He had undergone splenectomy 4 years earlier, and probably this component of the infiltrate which caused had received intermittent treatment with hydroxyurea. obstruction and which responded to chemotherapy. On admission he was taking fluoxetine and penicillin Extramedullary haematopoiesis at this site probably V. Examination revealed him to be hypertensive preceded infiltration by the myelofibrotic process. (170/90) with widespread lymphadenopathy ( left and Renal extramedullary haematopoiesis and myeloright inguinal, left axilla and left supraclavicular fibrosis is a rare, but treatable cause of renal nodes). Investigations showed Hb 8.1 g/dl, MCV 100 impairment. with a leucoerythroblastic film, plasma creatinine 198 mmol/l, urea 10 mmol/l and normal electrolytes. Lymph node and bone marrow biopsies showed myelofibrosis Renal ultrasound revealed normal sized kidneys of increased echogenicity consistent with an Suggested Reading infiltrative process. The patient subsequently underwent a renal biopsy, histology of which showed extramedullary haema1. Bartlett RP, Greipp PR, Tefferi A, Cupps RE, topoiesis and myelofibrosis infiltrating renal and periMullan BP, Trastek VF. Extramedullary haemarenal tissue (Figure 1). The patient subsequently topoiesis manifesting as a symptomatic pleural received chemotherapy with ‘FLAG’ (fludarabine, effusion. Mayo Clin Proc 1995; 70: 1161–116 cytarabine, arabinoside and G-CSF), with recovery of 2. Wat NM, Tse KK, Chan FL, Lam KS. Adrenal renal function (creatinine 80 mmol/l ) and blood presextramedullary haematopoiesis: diagnosis by a sure returning to normal. non-invasive method. Br J Haematology 1998; 100: 725–727 3. Holt SG, Field P, Carmichael P, Mehta A, Discussion Jarmulowicz M, Clarke D, Hilson A, Burns A. Extramedullary haematopoeisis in the renal parenExtramedullary haematopoiesis (EMH) is usually a chyma as a cause of acute renal failure in myelocomplication of chronic haematological conditions fibrosis. Nephrol Dial Transplant 1995; 10: such as myelofibrosis, and normally occurs in the 1438–1440 reticuloendothelial system ( liver, spleen and lymph 4. Gryspeerdt S, Oyen R, Van Hoe L, Baert AL, nodes), and less commonly in the pleura, pericardium, Boogaerts M. Extramedullary haematopoeisis adrenal glands and kidneys [1–3]. EMH is often encasing the pelvicalyceal system: CT findings. asymptomatic, may cause organomegaly and rarely Annal Haematol 1995; 71: 53–56 end-organ damage. 5. Moskovitz B, Malberger E, Brenner B, Gaitini D, Renal parenchymal EMH presenting as renal failure Vardi Y, Bolkier M. Renal extramedullary haemaresponsive to chemotherapy and radiotherapy has been topoeisis simulating hypernephroma. Eur Urol 1991; 19: 343–345 6. Wright, RER. Pararenal extramedullary haematoCorrespondence and offprint requests to: Dr Nick Woodward, poietic tissue. An unusual manifestation of myeloDepartment of Medicine, Worthing & Southlands Hospital NHS Trust, Lyndhurst Road, Worthing, West Sussex BN11 2DH, UK. fibrosis. Clin Radiol 1991; 44: 210–211