Taylor Ryan M, Bockenstedt Paula, Su Grace L, Marrero Jorge A, Pellitier Shawn M, Fontana Robert J
Division of Gastroenterology, University of Michigan, Ann Arbor, MI 48109-0362, USA.
Liver Transpl. 2006 May;12(5):781-91. doi: 10.1002/lt.20715.
Thrombocytopenia is common among liver transplant candidates and recipients. The aim of our study was to determine the incidence and outcome of new-onset immune-mediated thrombocytopenic purpura (ITP) following liver transplantation at a single center. Among the 256 liver transplant recipients with an International Classification of Diseases, Ninth Edition code for thrombocytopenia, 8 cases of new-onset ITP were identified, leading to an overall incidence of 0.7% in 1,105 consecutive liver transplant recipients over a 15-year period. All 8 patients were Caucasian, 5 (63%) were male, and the median age at ITP onset was 54 years (range, 15-63). The median platelet count at presentation was 3,500 cells/mL (range, 1,000-12,000) and liver disease was due to hepatitis C (38%), primary sclerosing cholangitis (38%), and cryptogenic cirrhosis (25%). The median time from transplant to ITP onset was 53.5 months (range, 1.9-173). Three of the 6 patients tested (50%) had cell-bound antiplatelet antibodies, 1 patient had an underlying hematological malignancy, and none of the organ donors had a history of ITP. Corticosteroids and/or immunoglobulin infusions were effective in 4 patients. However, serial rituximab infusions were required in 4 patients with persistent thrombocytopenia, and 3 of them eventually required splenectomy to induce disease remission. At a median follow-up of 19.7 months, 7 long-term survivors remain in remission with a median platelet count of 267,000 cells/mL. In conclusion, new-onset ITP is an infrequent but important cause of severe thrombocytopenia in liver transplant recipients. Corticosteroids and immunoglobulin infusions were effective in 50% while the remainder of patients required rituximab infusions or eventual splenectomy for long-term disease remission.
血小板减少症在肝移植候选者和接受者中很常见。我们研究的目的是确定在单一中心肝移植后新发免疫性血小板减少性紫癜(ITP)的发生率和结局。在256例患有第九版国际疾病分类血小板减少症编码的肝移植受者中,确诊了8例新发ITP,在15年期间的1105例连续肝移植受者中总体发生率为0.7%。所有8例患者均为白种人,5例(63%)为男性,ITP发病的中位年龄为54岁(范围15 - 63岁)。就诊时的中位血小板计数为3500个细胞/毫升(范围1000 - 12000),肝病病因包括丙型肝炎(38%)、原发性硬化性胆管炎(38%)和隐源性肝硬化(25%)。从移植到ITP发病的中位时间为53.5个月(范围1.9 - 173个月)。6例接受检测的患者中有3例(50%)存在细胞结合抗血小板抗体,1例患者有潜在血液系统恶性肿瘤,且器官供者均无ITP病史。皮质类固醇和/或免疫球蛋白输注对4例患者有效。然而,4例持续性血小板减少症患者需要连续输注利妥昔单抗,其中3例最终需要脾切除术以诱导疾病缓解。中位随访19.7个月时,7例长期存活者病情缓解,中位血小板计数为267000个细胞/毫升。总之,新发ITP是肝移植受者严重血小板减少症的一个少见但重要的原因。皮质类固醇和免疫球蛋白输注对50%的患者有效,而其余患者需要输注利妥昔单抗或最终进行脾切除术以实现长期疾病缓解。