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年轻患者的原发性血小板增多症。

Primary thrombocythemia in the young patient.

作者信息

Hoagland H C, Silverstein M N

出版信息

Mayo Clin Proc. 1978 Sep;53(9):578-80.

PMID:567265
Abstract

Primary thrombocythemia is typically a disease of older patients and frequently is associated with thrombosis and hemorrhage. Seven female and two male patients younger than 30 years were evaluated because of platelet counts exceeding 1 million/mm3. No hemorrhagic or thrombotic problems existed before diagnosis or developed during the follow-up period of 14 months to 10 years. Platelet function studies done in six of the nine patients showed absent aggregation with epinephrine and variable aggregation with adenosine diphosphate, as are seen in other chronic myeloproliferative states. One patient had spontaneous aggregation. Secondary causes of thrombocytosis were excluded. Only one patient received specific chemotherapy, which was given before 1973. Thus, thrombocythemia without a secondary cause appears to be much more benign in the young patient than in the older, and aggressive chemotherapy to lower the platelet count is not necessarily justified unless serious complications develop.

摘要

原发性血小板增多症通常是老年患者的疾病,常与血栓形成和出血相关。对9名年龄小于30岁的患者(7名女性和2名男性)进行了评估,这些患者的血小板计数超过100万/mm³。在诊断前或14个月至10年的随访期间均未出现出血或血栓形成问题。9名患者中的6名进行的血小板功能研究显示,对肾上腺素无聚集反应,对二磷酸腺苷有不同程度的聚集反应,这与其他慢性骨髓增殖性疾病所见相同。1名患者有自发聚集现象。排除了血小板增多症的继发性病因。只有1名患者接受了特异性化疗,该化疗于1973年之前进行。因此,无继发性病因的血小板增多症在年轻患者中似乎比老年患者更为良性,除非出现严重并发症,否则不一定有必要进行积极化疗以降低血小板计数。

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引用本文的文献

1
Primary thrombocythemia: diagnosis, clinical manifestations and management.
Ann Hematol. 1993 Aug;67(2):57-62. doi: 10.1007/BF01788127.
2
[Temperature-dependent spontaneous platelet aggregation in polycythemia vera and primary thrombocythemia: a diagnostic criterion].真性红细胞增多症和原发性血小板增多症中与温度相关的自发性血小板聚集:一项诊断标准
Klin Wochenschr. 1984 May 2;62(9):412-6. doi: 10.1007/BF01742298.
3
Painful extremities and neurological disorder in essential thrombocythaemia.原发性血小板增多症中的肢体疼痛和神经功能障碍。
J R Soc Med. 1984 May;77(5):372-4. doi: 10.1177/014107688407700507.
4
Myeloproliferative disease in children: a demographic study.儿童骨髓增殖性疾病:一项人口统计学研究。
J Clin Pathol. 1988 Aug;41(8):883-5. doi: 10.1136/jcp.41.8.883.
5
Thrombocytosis in young people: evaluation of 57 cases diagnosed before the age of 40.年轻人血小板增多症:对57例40岁前确诊病例的评估
Blut. 1990 Apr;60(4):233-7. doi: 10.1007/BF01728790.