Mitchell Oscar, Feldman David M, Diakow Marla, Sigal Samuel H
Department of Medicine, New York University School of Medicine, Langone Medical Center, New York, USA.
Department of Medicine, New York University School of Medicine, Langone Medical Center, New York, USA; Division of Gastroenterology and Liver Diseases, New York University School of Medicine, Langone Medical Center, New York, USA.
Hepat Med. 2016 Apr 15;8:39-50. doi: 10.2147/HMER.S74612. eCollection 2016.
Thrombocytopenia is the most common hematological abnormality encountered in patients with chronic liver disease (CLD). In addition to being an indicator of advanced disease and poor prognosis, it frequently prevents crucial interventions. Historically, thrombocytopenia has been attributed to hypersplenism, which is the increased pooling of platelets in a spleen enlarged by congestive splenomegaly secondary to portal hypertension. Over the past decade, however, there have been significant advances in the understanding of thrombopoiesis, which, in turn, has led to an improved understanding of thrombocytopenia in cirrhosis. Multiple factors contribute to the development of thrombocytopenia and these can broadly be divided into those that cause decreased production, splenic sequestration, and increased destruction. Depressed thrombopoietin levels in CLD, together with direct bone marrow suppression, result in a reduced rate of platelet production. Thrombopoietin regulates both platelet production and maturation and is impaired in CLD. Bone marrow suppression can be caused by viruses, alcohol, iron overload, and medications. Splenic sequestration results from hypersplenism. The increased rate of platelet destruction in cirrhosis also occurs through a number of pathways: increased shear stress, increased fibrinolysis, bacterial translocation, and infection result in an increased rate of platelet aggregation, while autoimmune disease and raised titers of antiplatelet immunoglobulin result in the immunologic destruction of platelets. An in-depth understanding of the complex pathophysiology of the thrombocytopenia of CLD is crucial when considering treatment strategies. This review outlines the recent advances in our understanding of thrombocytopenia in cirrhosis and CLD.
血小板减少症是慢性肝病(CLD)患者中最常见的血液学异常。它不仅是疾病进展和预后不良的指标,还常常妨碍关键的干预措施。从历史上看,血小板减少症一直被归因于脾功能亢进,即由于门静脉高压继发的充血性脾肿大导致脾脏中血小板的蓄积增加。然而,在过去十年中,我们对血小板生成的理解有了重大进展,这反过来又加深了我们对肝硬化中血小板减少症的认识。多种因素导致血小板减少症的发生,这些因素大致可分为导致血小板生成减少、脾脏扣押和破坏增加的因素。CLD中血小板生成素水平降低,加上直接的骨髓抑制,导致血小板生成率降低。血小板生成素调节血小板的生成和成熟,而在CLD中其功能受损。骨髓抑制可由病毒、酒精、铁过载和药物引起。脾脏扣押是由脾功能亢进导致的。肝硬化中血小板破坏率增加也通过多种途径发生:剪切应力增加、纤维蛋白溶解增加、细菌移位和感染导致血小板聚集率增加,而自身免疫性疾病和抗血小板免疫球蛋白滴度升高导致血小板的免疫性破坏。在考虑治疗策略时,深入了解CLD血小板减少症的复杂病理生理学至关重要。本综述概述了我们对肝硬化和CLD中血小板减少症认识的最新进展。