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[成人自身免疫性血小板减少症:诊断与治疗]

[Adult autoimmune thrombocytopenia: diagnosis and treatment].

作者信息

Lechner Klaus, Weltermann Ansgar, Pabinger Ingrid

机构信息

Abteilung Hämatologie/Hämostaseologie, Universitätsklinik für Innere Medizin I, Medizinische Universität Wien, Wien, Austria.

出版信息

Wien Klin Wochenschr. 2006 May;118(9-10):255-64. doi: 10.1007/s00508-006-0602-5.

Abstract

The incidence of AITP is 20-30/million/year. The diagnosis is based on the finding of an isolated thrombocytopenia without other blood abnormalities and absence of a palpable spleen. Additional tests such as bone marrow examination, determination of platelet antibodies and of thrombopoetin are required only in special cases. The usual first line therapy in patients with bleeding tendency and a low platelet count is prednisolone at a dose of 1 mg/kg/day. Patients who have platelet counts of less than 20,000/microl 3-6 months after steroid therapy are candidates for splenectomy, in particular if more than 0.1 mg/kg/day prednisolone is required to keep the patient free of bleedings. Laparoscopic splenectomy has a low mortality (0.2%) and morbidity (10%). The risk of post-splenectomy overwhelming pneumococcal septicaemia can be minimized by preoperative vaccination. Older patients, who have low platelet counts after splenectomy, have a high bleeding risk. The most effective treatment options for these patients are cyclophosphamide, azathioprine and rituximab, but the choice of treatment should be carefully considered, since the risk of adverse effects may be greater than the risk of fatal bleeding.

摘要

免疫性血小板减少性紫癜(AITP)的发病率为每年20 - 30/百万。诊断依据是发现单纯性血小板减少,无其他血液异常且脾脏不可触及。仅在特殊情况下才需要进行其他检查,如骨髓检查、血小板抗体及血小板生成素测定。对于有出血倾向且血小板计数低的患者,常用的一线治疗药物是泼尼松龙,剂量为1毫克/千克/天。接受类固醇治疗3 - 6个月后血小板计数低于20,000/微升的患者是脾切除术的候选者,特别是如果需要超过0.1毫克/千克/天的泼尼松龙来防止患者出血。腹腔镜脾切除术的死亡率低(0.2%),发病率也低(10%)。术前接种疫苗可将脾切除术后暴发性肺炎球菌败血症的风险降至最低。脾切除术后血小板计数低的老年患者出血风险高。对这些患者最有效的治疗选择是环磷酰胺、硫唑嘌呤和利妥昔单抗,但治疗选择应谨慎考虑,因为不良反应的风险可能大于致命性出血的风险。

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