Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease.
Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease.
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DOI：
10.1002/ajh.24224
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发表时间：
2016-02
影响因子：
12.8
通讯作者：
Brittain JE
中科院分区：
文献类型：
--
作者：
Anea CB;Lyon M;Lee IA;Gonzales JN;Adeyemi A;Falls G;Kutlar A;Brittain JE
越来越多的证据表明血小板在镰状细胞病（SCD）中的作用。尽管血小板具有促炎、闭塞的性质，但血小板在急性胸部综合征（ACS）中的作用仍未得到充分研究。为了提供证据并可能描述ACS与血小板之间假定联系的促成因素，我们对20例SCD病例进行了尸检研究-其中10例死于ACS，10例死亡与ACS无关。收集肺组织病理学和病史。我们发现10例ACS患者中有3例出现弥散性肺血小板血栓，而与之匹配的非ACS患者中无一例出现。那些检测到血栓的病例与内皮vWF的显著沉积和检测到粘附于内皮的大的vWF聚集体相关。潜在的临床危险因素是年龄较小和就诊时血小板计数较高。然而，我们也注意到，在每一个肺部有血小板血栓的病例中，死亡前血小板计数都有急剧和显著的下降。在这项研究中，无论是使用羟基脲还是死前输血都与血小板血栓无关。令人惊讶的是，在所有病例中，血栓性和增生性肺丛状病变均存在严重的肺动脉重塑。重塑的严重程度与严重的ACS病史或羟基脲使用无关，但与年龄呈负相关。因此，我们提供了证据证明存在血小板血栓的情况下，致命的ACS和描述的临床相关性。我们还提供了SCD中肺重构的新相关性。Am. J. Hematol. 91：173-178，2016.© 2015作者。Wiley Periodicals，Inc.出版的美国血液学杂志。
A growing body of evidence suggests a role for platelets in sickle cell disease (SCD). Despite the proinflammatory, occlusive nature of platelets, a role for platelets in acute chest syndrome (ACS), however, remains understudied. To provide evidence and potentially describe contributory factors for a putative link between ACS and platelets, we performed an autopsy study of 20 SCD cases—10 of whom died from ACS and 10 whose deaths were not ACS‐related. Pulmonary histopathology and case history were collected. We discovered that disseminated pulmonary platelet thrombi were present in 3 out of 10 of cases with ACS, but none of the matched cases without ACS. Those cases with detected thrombi were associated with significant deposition of endothelial vWF and detection of large vWF aggregates adhered to endothelium. Potential clinical risk factors were younger age and higher platelet count at presentation. However, we also noted a sharp and significant decline in platelet count prior to death in each case with platelet thrombi in the lungs. In this study, neither hydroxyurea use nor perimortem transfusion was associated with platelet thrombi. Surprisingly, in all cases, there was profound pulmonary artery remodeling with both thrombotic and proliferative pulmonary plexiform lesions. The severity of remodeling was not associated with a severe history of ACS, or hydroxyurea use, but was inversely correlated with age. We thus provide evidence of undocumented presence of platelet thrombi in cases of fatal ACS and describe clinical correlates. We also provide novel correlates of pulmonary remodeling in SCD. Am. J. Hematol. 91:173–178, 2016. © 2015 The Authors. American Journal of Hematology Published by Wiley Periodicals, Inc.