■原发性血小板增多症(ET)是一种罕见的慢性骨髓增殖性血液病,导致血小板计数升高。三分之二的患者在其一生中无症状,而其他人可能会出现红肿等症状,拥塞,和长时间无症状间隔后的红血病。
■作者介绍了一个罕见的55岁女性,尽管接受了阿司匹林和氯沙坦治疗,最终发展为数字坏疽。在进一步的工作中,她的血小板计数升高,JAK2突变阳性.她的血小板计数在阿司匹林治疗期间减少,羟基脲,还有肝素,接着她食指的坏死尖端被手术清创。
■显著症状,如严重的肢端紫癜,甚至是周围坏疽,可以用单剂量的阿司匹林治疗。每天服用阿司匹林,该病例发展为严重形式的ET。此外,而血小板增多症在理论上使患者容易发生血栓性并发症,几乎没有证据支持绝对血小板计数与血栓形成之间的相关性.
■ET的初始症状可能非常严重且不常见,尽管以前每天服用低剂量的阿司匹林,但仍可能发展成动脉血管血栓。
UNASSIGNED: Essential thrombocythemia (ET) is a rare chronic myeloproliferative hematologic disorder, leading to an elevated platelet count. Two-thirds of patients are asymptomatic during their lifetime, while others may experience symptoms like redness, congestion, and erythromelalgia after long symptom-free intervals.
UNASSIGNED: The authors present a rare instance of a 55-year-old female who, despite receiving aspirin and losartan treatment, eventually developed digital gangrene. In further work-ups, she had an elevated platelet count and a positive JAK 2 mutation. Her platelet count was reduced throughout treatment with aspirin, hydroxyurea, and heparin, which was followed by the necrotic tip of her index finger being surgically debrided.
UNASSIGNED: Significant symptoms, such as severe acrocyanosis and even peripheral gangrene, can be treated with a single dose of aspirin. Daily aspirin consumption withstanding, this
case developed the severe form of ET. In addition, while thrombocytosis predisposes patients to thrombotic complications in theory, there is little evidence to support a correlation between absolute platelet count and thrombosis.
UNASSIGNED: The initial symptom of ET could be such severe and uncommon that may develop arterial acral thrombosis despite previous daily low-dose aspirin consumption.