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年轻人血小板增多症:对57例40岁前确诊病例的评估

Thrombocytosis in young people: evaluation of 57 cases diagnosed before the age of 40.

作者信息

Randi M L, Fabris F, Girolami A

机构信息

University of Padua Medical School, Institute of Medical Semeiotics, Italy.

出版信息

Blut. 1990 Apr;60(4):233-7. doi: 10.1007/BF01728790.

Abstract

Over the past 13 years 57 cases of primary thrombocytosis in young people have been studied. Only patients with a platelet count over 500 x 10(9)/liter and a follow-up longer than 2 years were considered. Thrombocytosis in young people represents approximately 25% of total cases referred to our department during this period. The most common causes are essential thrombocythemia (20 cases) and secondary thrombocytosis (22 cases). The highest platelet counts are found in essential thrombocythemia patients. Most of our patients were discovered by a fortuitous hematological examination. In contrast, 5 out of the polycythemic patients were recognized after a thrombosis. The same was true for 2 out of 20 essential thrombocythemia subjects. Four subjects (2 essential thrombocythemia and 2 secondary thrombocytosis) were diagnosed after hemorrhages. The overall survival was very good except for leukemic patients and thrombocytosis secondary to neoplasms. Vascular complications after diagnosis were scarce: 2 polycythemia vera patients showed bleedings during antiaggregating therapy. None of our patients developed epithelial cancer, malignant lymphoma or myelofibrosis. Vascular traumata seem more frequent in polycythemia vera regardless of age. Therefore, it seems useful to treat polycythemic patients, while no therapy seems to be indicated in other forms of thrombocytosis.

摘要

在过去13年里,我们对57例青年原发性血小板增多症患者进行了研究。仅纳入血小板计数超过500×10⁹/升且随访时间超过2年的患者。青年血小板增多症约占同期转诊至我科病例总数的25%。最常见的病因是原发性血小板增多症(20例)和继发性血小板增多症(22例)。原发性血小板增多症患者的血小板计数最高。我们的大多数患者是通过偶然的血液学检查发现的。相比之下,5例真性红细胞增多症患者在发生血栓形成后才被确诊。20例原发性血小板增多症患者中有2例也是如此。4例患者(2例原发性血小板增多症和2例继发性血小板增多症)在出血后被诊断出来。除白血病患者和肿瘤继发的血小板增多症外,总体生存率非常好。诊断后的血管并发症很少见:2例真性红细胞增多症患者在抗聚集治疗期间出现出血。我们的患者均未发生上皮癌、恶性淋巴瘤或骨髓纤维化。无论年龄大小,血管创伤在真性红细胞增多症中似乎更为常见。因此,对真性红细胞增多症患者进行治疗似乎是有益的,而其他形式的血小板增多症似乎无需治疗。

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