妊娠合并血小板减少的病因及治疗

目的探讨妊娠合并血小板减少症的病因及治疗。方法对128例妊娠合并血小板减少患者的临床资料进行回顾性分析。结果单纯由妊娠引起的血小板减少91例(71.09%),特发性血小板减少性紫癜(ITP)引起11例(8.59%),合并肝脏疾病10例(7.81%),重度妊高征引起14例(10.94%),Rh血型不合及病毒感染各1例(各占0.78%)。对血小板(50×109/L者用强的松治疗,分娩前后使用血小板制剂,同时考虑剖宫产。结论妊娠期血小板减少是最常见的妊娠合并血小板减少症类型。有合并症的血小板减少程度严重,大多<70×109/L,半数ITP有临床症状。糖皮质激素是治疗严重血小板减少的有效手段,术前血小板仍<50×109/L可输注浓缩血小板。     

Thrombocytopenia After Isolated Limb or Hepatic Perfusions with Melphalan: The Risk of Heparin-Induced Thrombocytopenia

Background: Three cases of heparin-induced thrombocytopenia (HIT) were observed in patients undergoing isolated limb perfusion (ILP) with melphalan. This occurrence prompted the discontinuation of prophylactic postoperative heparin in ILP patients and its avoidance in patients undergoing isolated hepatic perfusion (IHP). The need to reassess these decisions led to a review of thrombocytopenia in both patient populations.Methods: Records of all patients treated with ILP or IHP at our institution from July 1992 through November 1996, were reviewed. Nine IHP patients were tested prospectively for heparinrelated antibodies using serum samples obtained perioperatively and during the second postoperative week.Results: Thrombocytopenia (<100,000 platelets/L) developed postoperatively in 30% of 131 ILP patients and in 77% of 56 IHP patients. No cases of HIT were identified other than the three who had been previously diagnosed. The prevalence of HIT in heparinized ILP patients was 2.8% (3/108). All nine IHP patients developed heparin-related antibodies postoperatively.Conclusions: Because the prevalence of HIT following ILP is in the range observed in other clinical settings, postoperative heparin prophylaxis is an option. However, it probably should be limited to the first week, and daily platelet counts should be reviewed for a pattern of thrombocytopenia consistent with HIT. The prevalence of heparin-related antibodies after IHP is so high that prophylactic heparin should be avoided in this setting.     

CONGENITAL ROSAI-DORFMAN DISEASE WITHOUT LYMPHADENOPATHY

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of γ-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.     

重组人白细胞介素-11治疗实体瘤化疗所致的血小板减少症的随机对照研究

目的:观察重组人白细胞介素-11(rhIL-11)治疗实体瘤化疗所致的血小板减少症的客观疗效;观察rhIL-11在人体的不良反应及其安全性.方法:本研究采用随机对照试验,55例化疗后血小板低于50×109/L的患者,随机分为A组和B组,A组接受rhIL-11,B组不接受rhIL-11治疗.主要观察IL-11能否治疗化疗引起的血小板减少症.结果:A组血小板值在第2～21d均高于B组,第4～21天差别具有显著的统计学意义;A组Ⅱ°、Ⅲ°及Ⅳ°血小板减少的持续天数分别为3.0d、3.2d及0.4d,B组分别为5.1d、5.8d及2.2d,A组血小板减少持续天数短于B组,但无统计学差异.IL-11的不良反应主要包括:心悸、心律失常、水肿、发热、关节肌肉疼痛、注射局部疼痛、皮疹、头痛头晕、乏力等.大多较轻,可以耐受.结论:rhIL-11能刺激血小板增生,治疗化疗引起的血小板降低,是一种有效、安全的治疗血小板减少的药物,值得进一步研究.     

妊娠合并血小板减少112例临床分析

目的:探讨妊娠合并血小板减少的发病机制及围生期的处理方法。方法:回顾分析天津医科大学总医院1992年至2002年间112例妊娠合并血小板减少患者的病因及临床处理经验。结果:112例孕妇中特发性血小板减少性紫癜27例,占24.1%;子痫前期44例,占39.3%;妊娠期特发性血小板减少40例,占34.8%;血栓性血小板减少性紫癜1例,占0.89%。阴道分娩27例,剖宫产85例。产后出血13例,产褥感染1例。结论:多种原因可以导致妊娠妇女血小板减少。如无产科指征,以阴道分娩为宜;血小板<50×109/L时,在血源充足时行剖宫产。不主张采用侵入性检查方法确定胎儿血小板水平。     

血液肿瘤化疗后血清可溶性白细胞介素-11水平的变化及其意义

目的 观察血液肿瘤化疗后血清可溶性白细胞介素-11(sIL-11)水平与血小板数量改变的关系,探索能维持血小板安全水平的sIL-11临界值,以指导临床治疗.方法 收集血液肿瘤患者化疗前后的血标本,测定sIL-11和血小板水平,观察两者变化间关系并进行统计学分析.结果 99例患者完成研究.化疗后患者的sIL-11水平逐步升高,第6天达到峰值后逐渐下降;血小板数量随时间逐步降低,第10天达到最低值,然后逐渐上升;在血小板达到最低之前sIL-11已达峰值;患者化疗后sIL-11越高,血小板数量越有可能维持于较高水平.根据血小板水半最低值将病例分为两组比较,具较高血小板组有较高sIL-11峰值.其sIL-11平均增长速度快,血小板达到最低值的时间较晚,高sIL-11峰值的病例较多.多元回归显示化疗后血小板低于临界值的影响因素有:sIL-11达到最大值的日平均增长速度和化疗第4天sIL-11低于2000Pg/ml.结论 血液肿瘤患者化疗后sIL-11水平与血小板数量的变化存在相关关系,可以通过测量sIL-11的变化来预测血小板数量的变化趋势.化疗第4天sIL-11<2000 Pg/ml的患者发生严重血小板减少的可能性大,建议给予rhIL-11治疗或输注血小板治疗.     

The genetics of platelet count and volume in humans

The last decade has witnessed an explosion in the depth, variety, and amount of human genetic data that can be generated. This revolution in technical and analytical capacities has enabled the genetic investigation of human traits and disease in thousands to now millions of participants. Investigators have taken advantage of these advancements to gain insight into platelet biology and the platelet’s role in human disease. To do so, large human genetics studies have examined the association of genetic variation with two quantitative traits measured in many population and patient based cohorts: platelet count (PLT) and mean platelet volume (MPV). This article will review the many human genetic strategies—ranging from genome-wide association study (GWAS), Exomechip, whole exome sequencing (WES), to whole genome sequencing (WGS)—employed to identify genes and variants that contribute to platelet traits. Additionally, we will discuss how these investigations have examined and interpreted the functional implications of these newly identified genetic factors and whether they also impart risk to human disease. The depth and size of genetic, phenotypic, and other -omic data are primed to continue their growth in the coming years and provide unprecedented opportunities to gain critical insights into platelet biology and how platelets contribute to disease.