진성다혈구증이 병발된 IgA 신증 1예 (A Case of Ig A Nephropathy Associatedwith Polycythemia Vera)

서지정보

· 발행기관 : 대한신장학회
· 수록지 정보 : Kidney Research and Clinical Practice / 21권 / 6호 / 1006 ~ 1010페이지
· 저자명 : 김지혜, 최바울, 정연순, 장성훈, 어완규, 천봉권, 임학

초록

이차성 적혈구증다증을 유발할 수 있는 원인으로는 신종양, 신낭종, 수신증, 신이식, 신동맥 협착증, Bartter씨 증후군 등이 알려져 있으며, 드문 원인으로 신증후군, 신경화증, 신우신염, 만성사구체신염과 막성사구체신염 등이 보고되어 있다1-4). 그러나 진성다혈구증과 동반된 사구체신염의 보고는 드물며5-7) 이들의 연관성은 아직 밝혀지지 않았다. 이에 저자들은 진성다혈구증에 병발된 IgA 신증을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

영어초록

Secondary polycythemia is occasionally associated with renal diseases such as renal tumors, cysts, hydronephrosis, renal transplantation, renal artery stenosis and Bartter's syndrome and is rarely associated with nephrotic syndrome, nephrosclerosis, pyelonephritis, chronic gromerulonephritis and membranous nephropathy. The association of polycythemia vera and Immunoglobulin A nephropathy (IgAN) is well not known, and there are only a few isolated reports presenting the concomitance of polycythemia vera and IgAN. We report one patient with concomitant polycythemia vera and Ig A nephropathy. A 53 year-old male visited our hospital because of elevated hemoglobin level. Blood value of hemoglobin was 22.1 g/dL. Isotopic blood studies with radioactive chromium (51Cr)-labelled red blood cells revealed a total blood volume of 90 mL/kg and total red cell volume of 61.8 mL/kg. The concentration of serum erythropoietin measured by radioimmunoassay was 14.29 mIU/mL (normal 10.2-25.2 mIU/mL). Bone marrow aspirate revealed hypercellularity and panmyelosis, characteristically. Renal biopsy specimens showed moderate mesangioproliferative lesions with mesangial IgA and C3 deposition. Treatment with phlebotomy, hydroxyurea and oral prednisolone (1 mg/kg/day) was done. There was no decrease of urinary protein following treatment of phlebotomy and hydroxyurea. But urinary protein decreased and hemoglobin level normalized following combination treatment of phlebotomy, hydroxyurea and oral prednisolone.

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