Gursoy A, Dogruk Unal A, Ayturk S, Karakus S, Nur Izol A, Bascil Tutuncu N, Guvener Demirag N
Department of Endocrinology and Metabolic Disease, Faculty of Medicine, Baskent University, 06490, Ankara, Turkey.
J Endocrinol Invest. 2006 Sep;29(8):742-4. doi: 10.1007/BF03344186.
A 39-yr-old man presented to our hospital with unexplained erythrocytosis and hypertension. His follow-up for erythrocytosis had begun 2 yr earlier in another hospital and he had been diagnosed with polycythemia rubra vera. On admission to our hospital he was hypertensive (165/95 mmHg) and, except for the presence of moon-like face and facial plethora, his physical examination was normal. His hemoglobin concentration was 19.2 g/dl, and hematocrit was 58.9% with an increased red blood cell mass of 58 ml/kg as measured by radioisotope (Cr51). Blood film, other hematological indices except for elevated leukocyte alkaline phosphatase score, arterial gas analysis, and examination of aspirated bone marrow were all normal. An abdominal ultrasonography showed no evidence of splenomegaly. A diagnosis of probable secondary erythrocytosis was made. Early-morning serum cortisol and 24-h urinary free cortisol concentration as well as serum ACTH were high. Serum cortisol was not suppressed by low-dose dexamethasone, but suppressed by high-dose dexamethasone. Pituitary magnetic resonance imaging showed no lesion. After inferior petrosal sinus sampling suggesting right-central ACTH secretion, the patient underwent transnasal-transsphenoidal pituitary adenomectomy. Both hypercortisolemia and erythrocytosis regressed completely after the adenomectomy. After the operation, the patient's hemoglobin concentration and hematocrit decreased steadily, and 1 month post-adenomectomy his hemoglobin is 14.9 g/dl and hematocrit 44.8%. Thus, Cushing's syndrome should be a routine part of evaluation of unexplained polycythemia.
一名39岁男性因不明原因的红细胞增多症和高血压前来我院就诊。他的红细胞增多症随访于2年前在另一家医院开始，当时被诊断为真性红细胞增多症。入院时他患有高血压（165/95 mmHg），除了满月脸和面部充血外，体格检查正常。他的血红蛋白浓度为19.2 g/dl，血细胞比容为58.9%，放射性同位素（Cr51）测量的红细胞量增加至58 ml/kg。血涂片、除白细胞碱性磷酸酶评分升高外的其他血液学指标、动脉血气分析以及骨髓穿刺检查均正常。腹部超声检查未发现脾肿大迹象。诊断为可能的继发性红细胞增多症。清晨血清皮质醇、24小时尿游离皮质醇浓度以及血清促肾上腺皮质激素（ACTH）均升高。血清皮质醇不受小剂量地塞米松抑制，但受大剂量地塞米松抑制。垂体磁共振成像未发现病变。经岩下窦采血提示右侧中央型ACTH分泌后，患者接受了经鼻-蝶窦垂体腺瘤切除术。腺瘤切除术后，高皮质醇血症和红细胞增多症均完全消退。术后，患者的血红蛋白浓度和血细胞比容稳步下降，腺瘤切除术后1个月，其血红蛋白为14.9 g/dl，血细胞比容为44.8%。因此，库欣综合征应作为不明原因红细胞增多症评估的常规部分。
