Hematology-Oncology Section, Department of Medicine, College of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK.
Hematology Am Soc Hematol Educ Program. 2018 Nov 30;2018(1):548-552. doi: 10.1182/asheducation-2018.1.548.
Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be recognized and appropriately addressed. Depression may be the most common disorder following recovery from TTP and may be the most important issue for the patient's quality of life. Severe or moderate depression has occurred in 44% of Oklahoma Registry patients. Recognition of depression by routine screening evaluations is essential; treatment of depression is effective. Minor cognitive impairment is also common. The recognition that cognitive impairment is related to the preceding TTP can provide substantial emotional support for both the patient and her family. Because TTP commonly occurs in young black women, the frequency of systemic lupus erythematosus, as well as other autoimmune disorders, is increased. Because there is a recognized association of TTP with pregnancy, there is always concern for subsequent pregnancies. In the Oklahoma Registry experience, relapse has occurred in only 2 of 22 pregnancies (2 of 13 women). The frequency of new-onset hypertension is increased. The most striking evidence for the impact of morbidities following recovery from TTP is decreased survival. Among the 77 patients who survived their initial episode of TTP (1995-2017), 16 (21%) have subsequently died, all before their expected age of death (median difference, 22 years; range 4-55 years). The conclusion from these observations is clear. Following recovery from TTP, multiple health problems occur and survival is shortened. Therefore, careful continuing follow-up is essential.
尽管获得性自身免疫性血栓性血小板减少性紫癜 (TTP) 康复后复发的风险可能是最大的担忧，但仍有许多其他重大健康问题需要认识并妥善处理。抑郁症可能是 TTP 康复后最常见的疾病，也是患者生活质量最重要的问题。在俄克拉荷马州登记处的患者中，有 44%出现严重或中度抑郁。通过常规筛查评估识别抑郁至关重要；治疗抑郁是有效的。轻度认知障碍也很常见。认知障碍与先前的 TTP 有关的认识可以为患者及其家属提供实质性的情感支持。由于 TTP 常见于年轻的黑人女性，系统性红斑狼疮以及其他自身免疫性疾病的发病率也有所增加。由于 TTP 与妊娠有明确的关联，因此始终会关注随后的妊娠。在俄克拉荷马州登记处的经验中，仅在 22 次妊娠中的 2 次（13 名女性中的 2 次）中复发。新发高血压的频率增加。在 TTP 康复后发生的多种疾病对生存的影响最明显的证据是生存时间缩短。在 77 名幸存其首次 TTP 发作的患者（1995-2017 年）中，有 16 名（21%）随后死亡，均早于预期死亡年龄（中位数差异为 22 岁；范围 4-55 岁）。从这些观察结果得出的结论很明确。TTP 康复后，会出现多种健康问题，生存时间缩短。因此，必须进行仔细的持续随访。
