课件：弥散性血管内凝血诊断和治疗进展.ppt

Alterations of DIC markers in two PS cases with giant hemangiomas ? Platelets APTT PT TT Fibrinogen AT D-dimer (×109/L) (s) (s) (s) (g/L) (%) (μg/L) ?Case 1 Before splenectomy 71 61.7 20.1 22.8 0.6 50 19.1 After splenectomy 110 37.2 14.4 18.2 2.91 83 3.22 Case 2 Before splenectomy 81 44.9 14.7 24.0 0.34 70.4 18.0 After splenectomy 138 40.1 14.5 18.2 3.06 98.6 1.95 Normal control 100-300 28-40 10.8-13.5 14.0-21.0 2.00-4.00 70-125 0.01-0.50? A scoring system for diagnozing Proteus syndrome Macrodactyly and/or hemihypertrophy 5 points Plantar or palmar cerebriform hyperplasia 4 points Lipomas/subcutaneous tumours 4 points Epidermal naevus 3 points Macrocephaly and/or skull exostosis 2.5 points miscellaneous other minor abnormalities 1 point A score of 13 or greater confirms its diagnosis. Our two patients scored 15.5 and 13 points, respectively, and met the criteria of Proteus syndrome diagnosis. 男，5岁，皮下大片瘀斑。 WBC 8.7×109/L，RBC 3.67×1012/L，Hb 132 g/L，Plt 19×109/L。初诊为ITP。 女，43岁，因卵巢囊肿住院手术。术后3天每天均有大血肿。APTT 86.7s，PT 120s，TT 15.8s，Fg 3.1g/L，血小板165×109/L。拟诊为DIC。追问病史，因10年前瓣膜置换每日服用华法林（近半年为3mg/d），未作监测。 晚期肝硬化的凝血改变 男，52岁，晚期肝硬化肝功能衰竭，牙龈出血，皮肤少量瘀斑。 APTT48.2s，PT31.4s，TT19.1s，纤维蛋白原0.87g/L，凝血酶原31%，因子Ⅴ42%，因子Ⅶ22.7%，因子Ⅷ104%，因子Ⅸ64%，因子Ⅹ37.8%，Ⅺ56.6%。 VOD 女，22岁。ALL化疗后复发3次，接受来自母亲的半相合骨髓移植。术后第5天出现腹痛、腹胀、黄疸与肝肿大；体重每日增加10斤。全身皮肤粘膜广泛出血。 T-BIL 84.5μmol/L，ALT 1820 U/L， AST 2670U/L，LDH 2880 U/L。APTT 45.4s，PT 35.4s，TT 23.4s，Fg 1.03g/L，AT 38%，D二聚体3.18 mg/L，FⅤ:C 18%，FⅦ:C 4%，FⅧ:C 91%，FⅨ:C 23%，ADAMTS13 100% Murine coagulation factor VIII is synthesized in endothelial cells The primary cellular source o