Polycythemia vera Presenting with Rapidly Progressive Glomerulonephritis and Pyoderma gangrenosum
Polycythemia vera Presenting with Rapidly Progressive Glomerulonephritis and Pyoderma gangrenosum
复制标题
真性红细胞增多症表现为急进性肾小球肾炎和坏疽性脓皮病
作者：
O. Oymak;F. S. Oymak;T. Patiroğlu;H. Taskapan;A. Doğukan;M. Çetin;C. Utaş
尊敬的先生，真性红细胞增多症(PV)是一种以红细胞增多症、白细胞增多症和血小板增多症为特征的骨髓增生性疾病。血栓事件是发病率和死亡率的重要原因[1]。与PV相关的各种副肿瘤性疾病和免疫介导性疾病，如坏疽脓皮病、Sweet综合征、白细胞破碎性血管炎、自身免疫性溶血性贫血和溃疡性结肠炎已被描述[2-6]。本文报告一位因快速进行性肾小球肾炎(RPGN)而患坏疽脓皮病并发展为急性肾功能衰竭的PV患者。1例66岁男性患者因肾功能不全、尿沉渣活跃而转诊至医院。在过去的两周里，他一直遭受虚弱、头晕和弥漫性腹痛的痛苦。体格检查，轻度高血压(160/100毫米汞柱)，面色发红，结膜注射，中度肝脾肿大。双腿有大(8！10厘米)溃烂皮损，中央塌陷，皮损周围有红斑，提示坏疽脓皮病。入院时实验室检查：血红蛋白20.8g/dl，红细胞压积70%，白细胞17.9B/109/L，血小板660,109/L，血尿素氮128 mg/dl，血肌酐7.5 mg/dl，尿酸18.5 mg/dl，总蛋白7.9g/dl，白蛋白3.6g/dl。补体成分C3和C4水平分别为58和44 mg/dl。尿检显示蛋白尿(300 mg/dl)，镜检见高倍镜下可见大量红细胞和3-4个颗粒铸型。动脉血气：PH7.25，PO2 92 mm Hg，PCO2 24 mm Hg，HCO3 14.7mEq/L，O2饱和度96%。血清促红细胞生成素水平较低，为2mU/ml(正常范围4~25)。腹部超声显示肝脾肿大，双侧肾肿大(双肾长15.8厘米)。这些发现提示为PV，可能与急性肾功能衰竭有关。立即行肾活检。12个肾小球均显示上皮细胞、内皮细胞和系膜细胞增殖，轻度基底膜增厚，局灶性坏死区，细胞新月体和炎性细胞浸润，与RPGN一致(图3)。1)。免疫组织化学检测显示免疫球蛋白、补体COM呈阳性。
Dear Sir, Polycythemia rubra vera (PV) is a myeloproliferative disease characterized by polycythemia, leukocytosis, and thrombocytosis. Thromboembolic events are important causes of morbidity and mortality [1]. Various paraneoplastic and immunologically mediated diseases associated with PV such as pyoderma gangrenosum, Sweet’s syndrome, leukocytoclastic vasculitis, autoimmune hemolytic anemia, and ulcerative colitis have been described [2–6]. Here, a patient with PV who had pyoderma gangrenosum and developed acute renal failure due to rapidly progressive glomerulonephritis (RPGN) is presented. A 66-year-old male patient was referred to the hospital because of renal dysfunction with an active urinary sediment. He has suffered from weakness, dizziness, and diffuse abdominal pain for the last 2 weeks. On physical examination, he had mild hypertension (160/100 mm Hg), a reddish face, injected conjunctivae, and moderate hepatosplenomegaly. There were large (8! 10 cm) ulcerated skin lesions on both legs which showed central slough and perilesional erythema, suggesting pyoderma gangrenosum. Laboratory studies on admission were: hemoglobin 20.8 g/dl, hematocrit 70% white blood cell count 17.9 B 109/l, platelet count 660! 109/l, blood urea nitrogen 128 mg/dl, serum creatinine 7.5 mg/dl, uric acid 18.5 mg/dl, total protein 7.9 g/dl, and albumin 3.6 g/dl. Complement component C3 and C4 levels were 58 and 44mg/dl, respectively. Urinalysis revealed proteinuria (300 mg/dl), and many erythrocytes and 3–4 granular casts per high-power area were seen on microscopic examination. Arterial blood gases were: pH 7.25, PO2 92 mm Hg, PCO2 24 mm Hg, HCO–3 14.7 mEq/l, and O2 saturation 96%. The serum erythropoietin level was low with 2 mU/ml (normal range 4–25). Abdominal ultrasonography showed hepatosplenomegaly and bilateral nephromegaly (both kidneys measured 15! 8 cm). These findings were suggestive of PV, possibly associated with acute renal failure. Renal biopsy was performed immediately. All of 12 glomeruli showed epithelial, endothelial, and mesangial cellular proliferation, mild basal membrane thickening, focal necrosis areas, cellular crescents, and infiltration of inflammatory cells, consistent with RPGN (fig. 1). Immunohistochemical studies revealed positive staining for IgG, complement com-