Iyengar Abhinav, Sheppard Dawn
Hematology Division, Department of Medicine, The Ottawa Hospital, 501 Smyth Road, Ottawa, ON, Canada K1H 8L6.
Case Rep Hematol. 2013;2013:615189. doi: 10.1155/2013/615189. Epub 2013 Nov 7.
A previously healthy 79-year-old female was referred to hematology for further evaluation of erythrocytosis. Two years earlier she had been diagnosed with ER/PR-positive ductal carcinoma of the breast and was receiving hormonal therapy with exemestane. No secondary cause of erythrocytosis was identified. Serum erythropoietin (EPO) level was normal, and molecular testing for the JAK2 V617F and exon 12 mutations was negative. A bone marrow biopsy showed a mild increase in erythropoiesis, and no spontaneous erythroid colonies were demonstrated. Erythrocytosis is common reason for referral to a hematologist. The myeloproliferative disorder, polycythemia vera, and the rare congenital polycythemias represent primary erythrocytosis. Common secondary causes include smoking, obstructive sleep apnea, and other pulmonary diseases. Erythrocytosis is well described with certain classes of drugs, including androgens. We hypothesize that exemestane contributed to the development of erythrocytosis in our patient. To our knowledge, erythrocytosis has not been previously described in association with aromatase inhibitors. These drugs prevent the conversion of androstenedione and testosterone to estrogen; thus the physiologic mechanisms may be similar to those responsible for erythrocytosis seen with exogenous androgens. These mechanisms are not well understood, but may include altered iron metabolism by a reduction in hepcidin levels.
一名既往健康的79岁女性因红细胞增多症被转诊至血液科进行进一步评估。两年前,她被诊断为雌激素受体/孕激素受体(ER/PR)阳性的乳腺导管癌,正在接受依西美坦激素治疗。未发现红细胞增多症的继发原因。血清促红细胞生成素(EPO)水平正常,JAK2 V617F和第12外显子突变的分子检测为阴性。骨髓活检显示红细胞生成轻度增加,未发现自发红系集落。红细胞增多症是转诊至血液科医生的常见原因。骨髓增殖性疾病、真性红细胞增多症和罕见的先天性红细胞增多症代表原发性红细胞增多症。常见的继发原因包括吸烟、阻塞性睡眠呼吸暂停和其他肺部疾病。某些类别的药物(包括雄激素)也会导致红细胞增多症。我们推测依西美坦导致了我们患者红细胞增多症的发生。据我们所知,以前尚未有过依西美坦与红细胞增多症相关的报道。这些药物可阻止雄烯二酮和睾酮转化为雌激素;因此其生理机制可能与外源性雄激素导致红细胞增多症的机制相似。这些机制尚不完全清楚,但可能包括通过降低铁调素水平改变铁代谢。