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真性红细胞增多症——向骨髓纤维化的转化及随后的逆转。

Polycythaemia vera-transformation to myelofibrosis and subsequent reversal.

作者信息

Pettit J E, Lewis S M, Goolden A W

出版信息

Scand J Haematol. 1978 Jan;20(1):63-9. doi: 10.1111/j.1600-0609.1978.tb01555.x.

DOI:10.1111/j.1600-0609.1978.tb01555.x
PMID:625632
Abstract

A woman aged 67 who developed polycythaemia vera 21 years ago has been seen regularly since the diagnosis was first made. She was treated initially with pyrimethamine and then for 10 years by repeated venesections. After a 4 year period when her myeloproliferative disease was considered to be transitional a complete transformation to myelofibrosis occurred. She was treated by splenic irradiation and later with alkylating agents in an attempt to give her symptomatic relief from massive splenomegaly. Repeated and massive haemorrhage from gastro-oesophageal varices was treated initially by percutaneous transhepatic sclerosis of the gastro-oesophageal collateral vessels. A subsequent dacron mesocaval jump graft operation was successful in preventing further haematemesis and melaena. During the last year transformation back to polycythaemia vera has occurred. The evolution of her myeloproliferative disease has been fully documented by detailed blood counting, bone marrow aspirates and trephine biopsies, blood volume studies and other radioisotope investigations including the quantitation of functional erythropoietic tissue with 52Fe.

摘要

一名67岁女性,21年前被诊断为真性红细胞增多症,自确诊以来一直定期接受观察。她最初接受了嘧啶治疗,随后10年通过反复放血进行治疗。在经历了4年被认为是骨髓增殖性疾病过渡期后,病情完全转变为骨髓纤维化。她接受了脾脏放疗,后来使用烷化剂治疗,试图缓解因巨大脾肿大引起的症状。胃食管静脉曲张反复大量出血最初通过经皮肝穿刺硬化胃食管侧支血管进行治疗。随后进行的涤纶中腔静脉搭桥手术成功地防止了进一步的呕血和黑便。在过去一年中,病情又转变回真性红细胞增多症。通过详细的血细胞计数、骨髓穿刺和活检、血容量研究以及其他放射性同位素检查,包括用52Fe定量功能性造血组织,对她的骨髓增殖性疾病的演变过程进行了全面记录。

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Polycythaemia vera-transformation to myelofibrosis and subsequent reversal.真性红细胞增多症——向骨髓纤维化的转化及随后的逆转。
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Polycythaemia vera and myelofibrosis.真性红细胞增多症和骨髓纤维化。
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Transition of myelofibrosis to polycythaemia vera.骨髓纤维化向真性红细胞增多症的转变。
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Progression of primary myelofibrosis to polycythemia vera: A case report.原发性骨髓纤维化进展为真性红细胞增多症:一例报告。
Medicine (Baltimore). 2017 Jul;96(28):e7464. doi: 10.1097/MD.0000000000007464.
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Chorea with transitional myeloproliferative disease.伴有过渡性骨髓增殖性疾病的舞蹈症
J R Soc Med. 1985 Oct;78(10):864-6. doi: 10.1177/014107688507801017.