Department of Hematology, Kanazawa University Hospital, Takaramachi 13-1, Kanazawa City 920-8640, Ishikawa, Japan.
Int J Mol Sci. 2024 May 1;25(9):4956. doi: 10.3390/ijms25094956.
Platelets play an important role in hemostasis, and a low platelet count usually increases the risk of bleeding. Conditions in which thrombosis occurs despite low platelet counts are referred to as thrombosis with thrombocytopenia syndrome, including heparin-induced thrombocytopenia, vaccine-induced immune thrombotic thrombocytopenia, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, thrombotic microangiopathy (TMA), and disseminated intravascular coagulation. TMA includes thrombotic thrombocytopenic purpura, Shiga toxin-producing -associated hemolytic uremic syndrome (HUS), and atypical HUS. Patients with these pathologies present with thrombosis and consumptive thrombocytopenia associated with the activation of platelets and the coagulation system. Treatment varies from disease to disease, and many diseases have direct impacts on mortality and organ prognosis if therapeutic interventions are not promptly implemented. Underlying diseases and the results of physical examinations and general laboratory tests as part of a thorough workup for patients should promptly lead to therapeutic intervention before definitive diagnosis. For some diseases, the diagnosis and initial treatment must proceed in parallel. Utilization of not only laboratory tests but also various scoring systems is important for validating therapeutic interventions based on clinical information.
血小板在止血中发挥重要作用，血小板计数低通常会增加出血风险。尽管血小板计数低但仍发生血栓形成的情况称为伴有血小板减少的血栓形成综合征，包括肝素诱导的血小板减少症、疫苗诱导的免疫性血栓性血小板减少症、阵发性夜间血红蛋白尿、抗磷脂综合征、血栓性微血管病（TMA）和弥散性血管内凝血。TMA 包括血栓性血小板减少性紫癜、产志贺毒素相关溶血尿毒综合征（HUS）和非典型 HUS。这些病理患者表现为血栓形成和消耗性血小板减少症，伴有血小板和凝血系统的激活。治疗因疾病而异，如果不及时进行治疗干预，许多疾病会直接影响死亡率和器官预后。基础疾病以及体格检查和一般实验室检查的结果作为患者全面检查的一部分，应在明确诊断前及时进行治疗干预。对于某些疾病，诊断和初始治疗必须同时进行。不仅要利用实验室检查，还要利用各种评分系统，根据临床信息验证治疗干预的有效性非常重要。
