Morbidity and mortality in adults with idiopathic thrombocytopenic purpura
Morbidity and mortality in adults with idiopathic thrombocytopenic purpura
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DOI：
10.1182/blood.v97.9.2549
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发表时间：
2001-05-01
期刊：
影响因子：
20.3
通讯作者：
Brand, A
中科院分区：
文献类型：
--
作者：
Portielje, JEA;Westendorp, RGJ;Brand, A
为了研究成人特发性血小板减少性紫癜（ITP）的结局，我们对152例连续患者进行了随访研究，这些患者根据明确的算法进行治疗。长期结果是相对于诊断后2年的反应确定的，因为大多数（93%）最终达到血小板计数高于30.0 × 10（9）/L（30 000/穆尔）的患者是在这一时间范围内达到的。可对99%的患者进行死亡率和95%的患者进行发病率的完整随访，平均随访时间为10.5年。诊断后2年内，4名患者死亡，2名患者失访，12名患者被重新分类为继发性免疫性血小板减少症。在其余134例患者中，114例（85%）的血小板计数高于30.0 × 10（9）/L，而所有治疗均已停止。这些患者的长期死亡风险与一般人群相同。134例患者中有12例（9%）患有难治性疾病，死亡风险为4.2（95%置信区间，1.7-10.0），均患有重度血小板减少症。出血和感染同样导致这些患者的死亡。另外8例患者（6%）在维持治疗期间血小板计数高于30.0 × 10（9）/L。与难治性疾病患者相似，后者患者ITP相关住院率显著增加，但死亡率仅略高于一般人群。总之，大多数成人ITP患者预后良好，住院率低，死亡率低。中度血小板减少症患者未发生大体发病率和死亡率，这支持临床实践中避免进一步治疗。（Blood，2001;97：2549-2554）（C）2001年美国血液学会。
To study outcomes of adults with idiopathic thrombocytopenic purpura (ITP), we performed a follow-up study in a cohort of 152 consecutive patients who were treated according to a well-defined algorithm. Long-term outcomes were determined relative to the response 2 years after diagnosis, be cause most (93%) patients who ultimately attained platelet counts above 30.0 x 10(9)/L (30 000/muL) did so within this time frame. Complete follow-up for mortality could be studied in 99% of patients and for morbidity in 95% of patients,with a mean of 10.5 years. Within 2 years after diagnosis, 4 patients died, 2 were lost to follow-up, and 12 were reclassified as having secondary immune thrombocytopenia. Of the remaining 134 patients, 114 (85%) had obtained platelet counts above 30.0 x 10(9)/L while all therapies had been discontinued. These patients had a long-term mortality risk equal to the general population. Twelve of 134 patients (9%), all with severe thrombocytopenia, had refractory disease and suffered a mortality risk of 4.2 (95% confidence interval, 1.7-10.0). Bleeding and infection equally contributed to the death of these patients. Another 8 patients (6%) had platelet counts above 30.0 x 10(9)/L while on maintenance therapy. Similar to patients with refractory disease, these latter patients had considerably increased ITP-related hospital admissions, but mortality was only slightly higher than in the general population. In conclusion, most adults with ITP have a good outcome with infrequent hospital admissions and no excess mortality. The absence of gross morbidity and mortality in patients with moderate thrombocytopenia supports clinical practice refraining from further treatment. (Blood, 2001;97:2549-2554) (C) 2001 by The American Society of Hematology.