Parashar Yatendra, Kushwaha Rashmi, Kumar Ashutosh, Agarwal Kamal, Singh U S, Jain Mili, Verma S P, Tripathi A K
Junior Resident, Department of Pathology, King George's Medical University , Lucknow, Uttar Pradesh, India .
Associate Professor, Department of Pathology, King George's Medical University , Lucknow, Uttar Pradesh, India .
J Clin Diagn Res. 2016 Nov;10(11):EC01-EC04. doi: 10.7860/JCDR/2016/19985.8840. Epub 2016 Nov 1.
Patients of MPN commonly present with abnormalities in laboratory coagulation tests that are consistent with hypercoagulable state. Some individuals with MPN exhibit a pattern of exclusive bleeding or thrombotic events; many others have both bleeding and thrombosis during the course of the disease.
This study was undertaken to assess the haemostatic defects and platelet functions in patients of MPN.
One year prospective study was conducted at a tertiary care centre in North India in Department of Pathology in collaboration with Department of Clinical Haematology. All recently diagnosed cases of MPN along with 30 age and sex matched controls were included. Patients on antiplatelet drugs, antimyeloproliferative treatment, vitamin K agonists or antagonists, OCPs, Platelet count <1,00,000/μl, high grade fever, liver disease, pregnancy were excluded from this study. All the patients underwent screening investigations like CBC, peripheral smear evaluation, BT, PT, aPTT, Protein C and S measurement (clot based assay) and aggregation studies with ADP (5μM) (Optical Aggregometry with AGGRO/LINK 8 software and CHRONOLOG 700 aggregometer).
In present study, 50 cases were included. There was an occult prothrombotic state, suggested by significantly (p<0.001) reduced levels of Protein C and Protein S, but no patient presented with frank thrombosis while 8 out of 50 patients had haemorrhagic manifestations ranging from subdural haematoma to pin point petechial haemorrhages. Patients of CML-CP, ET, PV, PMF, MPN-NOS showed significantly reduced maximal aggregation with ADP (5μM) when compared to control (p<0.001). MPV also showed a statistically significant increase in these patients.
Thrombohaemorrhagic complications significantly affect the morbidity and mortality of MPN patients. This can be assessed by the use of platelet aggregation studies, Protein C and S activities and other coagulation studies. Timely diagnosis of these prothrombotic/haemorrhagic states can decrease the morbidity in these patients.
骨髓增殖性肿瘤(MPN)患者通常在实验室凝血检查中出现与高凝状态相符的异常。一些MPN患者表现为单纯出血或血栓形成事件的模式;许多其他患者在疾病过程中既有出血又有血栓形成。
本研究旨在评估MPN患者的止血缺陷和血小板功能。
在印度北部一家三级医疗中心的病理科与临床血液学系合作进行了为期一年的前瞻性研究。纳入所有近期诊断的MPN病例以及30名年龄和性别匹配的对照。本研究排除正在接受抗血小板药物治疗、抗骨髓增殖治疗、维生素K激动剂或拮抗剂、口服避孕药、血小板计数<100,000/μl、高热、肝病、妊娠的患者。所有患者均接受了全血细胞计数(CBC)、外周血涂片评估、出血时间(BT)测定、凝血酶原时间(PT)测定、活化部分凝血活酶时间(aPTT)测定、蛋白C和蛋白S测定(基于凝血块的检测方法)以及用5μM二磷酸腺苷(ADP)进行的聚集试验(使用AGGRO/LINK 8软件和CHRONOLOG 700聚集仪进行光学聚集测定)等筛查检查。
在本研究中,共纳入50例病例。蛋白C和蛋白S水平显著降低(p<0.001)提示存在隐匿性血栓前状态,但无患者出现明显血栓形成,而50例患者中有8例有出血表现,从硬膜下血肿到针尖样瘀点出血不等。与对照组相比,慢性粒细胞白血病慢性期(CML-CP)、原发性血小板增多症(ET)、真性红细胞增多症(PV)、原发性骨髓纤维化(PMF)、未分类的骨髓增殖性肿瘤(MPN-NOS)患者用5μM ADP刺激时最大聚集率显著降低(p<0.001)。这些患者的平均血小板体积(MPV)也有统计学意义的升高。
血栓出血并发症显著影响MPN患者的发病率和死亡率。这可以通过血小板聚集试验、蛋白C和蛋白S活性以及其他凝血检查来评估。及时诊断这些血栓前/出血状态可降低这些患者的发病率。