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[Hemoglobin J Amiens beta 17 (A 14) Lys replaced by Asn. Coincidence of a functionally silent new abnormal hemoglobin and a polycythemia vera (author's transl)].

作者信息

Elion J, Wajcman H, Belkhodja-Dunda O, Lapoumeroulie C, Labie D, Messerschmitt J, Staal A M, Desablens B

出版信息

Nouv Rev Fr Hematol (1978). 1979;21(4):347-52.

PMID:121938
Abstract

A new abnormal hemoglobin, Hb J Amiens beta 17 (A 14) Lys replaced by Asn, has been discovered during the exploration of a recent polycythemia in a 65-year-old patient of Spanish extraction. Oxygen affinity of washed red blood cells was found to be normal at pH 7.13 (P 50 = 30.0 mmHg, N = 29.5 +/- 1). Cooperativity is unchanged, and no instability was detected. From this study, it is concluded that there is no relation between this functionally silent hemoglobin and the polycythemia. In fact, the recent appearance of the polycythemia, the involvement of the other blood cell lines, particularly the thrombocytosis, the high score of leukocyte alkaline phosphatases, and the results of the bone marrow biopsy led to the diagnosis of polycythemia vera.

摘要

相似文献

1
[Hemoglobin J Amiens beta 17 (A 14) Lys replaced by Asn. Coincidence of a functionally silent new abnormal hemoglobin and a polycythemia vera (author's transl)].
Nouv Rev Fr Hematol (1978). 1979;21(4):347-52.
2
Hemoglobin Villejuif [beta 123(H1) Thr----Ile]: a new variant found in coincidence with polycythemia vera.
Am J Hematol. 1989 Dec;32(4):294-7. doi: 10.1002/ajh.2830320410.
3
Polycythemia produced by hemoglobin Osler (beta-145 (HC2) Tyr yields Asp).由血红蛋白奥斯勒(β-145(HC2)酪氨酸产生天冬氨酸)引起的红细胞增多症。
Johns Hopkins Med J. 1975 Mar;136(3):132-6.
4
Polycythemia: a disease of all ages.红细胞增多症:一种各年龄段均可发生的疾病。
Geriatrics. 1974 Oct;29(10):124-34.
5
[Clinical studies of the erythrocytosis produced by a new hemoglobin with oxygen affinity : Hb Hôtel-Dieu alpha 2A beta 2 Asp replaced by Gly (author's transl)].一种具有氧亲和力的新型血红蛋白(Hb Hôtel-Dieu α2Aβ2 天冬氨酸被甘氨酸取代)所致红细胞增多症的临床研究(作者译)
Ann Med Interne (Paris). 1981;132(4):225-8.
6
Hemoglobin Syracuse (alpha2beta2-143(H21)His leads to Pro), a new high-affinity variant detected by special electrophoretic methods. Observations on the auto-oxidation of normal and variant hemoglobins.血红蛋白锡拉丘兹(α2β2-143(H21)组氨酸突变为脯氨酸),一种通过特殊电泳方法检测到的新型高亲和力变体。关于正常和变体血红蛋白自动氧化的观察。
J Clin Invest. 1975 Mar;55(3):469-77. doi: 10.1172/JCI107953.
7
[Clinical and biological studies of an hybrid S/Stanleyville II hemoglobin (alpha 2 78 Asn replaced by Lys beta 2 6 Glu replaced by Val) (author's transl)].
Nouv Rev Fr Hematol (1978). 1980;22(3):235-41.
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[Polycythemia resulting from abnormal hemoglobin with increased affinity for oxygen. Two cases (author's transl)].因血红蛋白异常对氧亲和力增加所致的红细胞增多症。两例(作者译)
Nouv Presse Med. 1980 Jan 26;9(5):285-9.
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Hemoglobin Barcelona beta 94 (FG1) Asp leads to His : a new hemoglobin Variant with increased oxygen affinity.血红蛋白巴塞罗那β94(FG1)天冬氨酸突变为组氨酸:一种具有增加氧亲和力的新型血红蛋白变体。
Nouv Rev Fr Hematol (1978). 1981;23(5):267-73.
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Endogenous erythroid colony assay in patients with polycythemia vera and its clinical significance.真性红细胞增多症患者内源性红系集落测定及其临床意义。
Chin Med J (Engl). 2004 May;117(5):668-72.

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