中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2014年
7期
633-636
,共4页
刘文洁%宣旻%薛峰%杨仁池
劉文潔%宣旻%薛峰%楊仁池
류문길%선민%설봉%양인지
因子Ⅹ缺乏%多发性骨髓瘤%颅内出血%治疗结果
因子Ⅹ缺乏%多髮性骨髓瘤%顱內齣血%治療結果
인자Ⅹ결핍%다발성골수류%로내출혈%치료결과
Factor Ⅹ deficiency%Multiple myeloma%Intracranial hemorrhages%Treatment outcome
目的 加深对获得性凝血因子Ⅹ缺乏症的认识.方法 对3例获得性凝血因子Ⅹ缺乏症患者的临床资料进行分析,并复习相关文献.结果 例1,男,57岁,诊断为多发性骨髓瘤轻链型、继发性淀粉样变、获得性凝血因子Ⅹ缺乏症,表现为自发性皮肤黏膜出血,凝血因子Ⅹ活性(FⅩ∶C)1.8％,予以MP(马法兰+曲安西龙)方案联合沙利度胺及对症治疗,FⅩ∶C未见升高,因原发病进展死亡.例2,男,41岁,以颅内出血入院,FⅩ∶C 26.8％,予以补充叶酸、维生素B12、维生素K,并输注红细胞、血小板及新鲜冰冻血浆治疗,颅内出血好转.例3,女,63岁,因反复发作四肢关节出血4个月入院,FⅩ∶C 6.1％,给予凝血酶原复合物、甲泼尼龙、硫唑嘌呤、利妥昔单抗治疗,FⅩ∶C未见明显升高,关节腔出血仍反复发作.结论 获得性凝血因子Ⅹ缺乏症临床表现具有异质性,诊断依赖病史和实验室检查,治疗包括控制出血和治疗原发病,预后与患者基础疾病相关.
目的 加深對穫得性凝血因子Ⅹ缺乏癥的認識.方法 對3例穫得性凝血因子Ⅹ缺乏癥患者的臨床資料進行分析,併複習相關文獻.結果 例1,男,57歲,診斷為多髮性骨髓瘤輕鏈型、繼髮性澱粉樣變、穫得性凝血因子Ⅹ缺乏癥,錶現為自髮性皮膚黏膜齣血,凝血因子Ⅹ活性(FⅩ∶C)1.8％,予以MP(馬法蘭+麯安西龍)方案聯閤沙利度胺及對癥治療,FⅩ∶C未見升高,因原髮病進展死亡.例2,男,41歲,以顱內齣血入院,FⅩ∶C 26.8％,予以補充葉痠、維生素B12、維生素K,併輸註紅細胞、血小闆及新鮮冰凍血漿治療,顱內齣血好轉.例3,女,63歲,因反複髮作四肢關節齣血4箇月入院,FⅩ∶C 6.1％,給予凝血酶原複閤物、甲潑尼龍、硫唑嘌呤、利妥昔單抗治療,FⅩ∶C未見明顯升高,關節腔齣血仍反複髮作.結論 穫得性凝血因子Ⅹ缺乏癥臨床錶現具有異質性,診斷依賴病史和實驗室檢查,治療包括控製齣血和治療原髮病,預後與患者基礎疾病相關.
목적 가심대획득성응혈인자Ⅹ결핍증적인식.방법 대3례획득성응혈인자Ⅹ결핍증환자적림상자료진행분석,병복습상관문헌.결과 례1,남,57세,진단위다발성골수류경련형、계발성정분양변、획득성응혈인자Ⅹ결핍증,표현위자발성피부점막출혈,응혈인자Ⅹ활성(FⅩ∶C)1.8％,여이MP(마법란+곡안서룡)방안연합사리도알급대증치료,FⅩ∶C미견승고,인원발병진전사망.례2,남,41세,이로내출혈입원,FⅩ∶C 26.8％,여이보충협산、유생소B12、유생소K,병수주홍세포、혈소판급신선빙동혈장치료,로내출혈호전.례3,녀,63세,인반복발작사지관절출혈4개월입원,FⅩ∶C 6.1％,급여응혈매원복합물、갑발니룡、류서표령、리타석단항치료,FⅩ∶C미견명현승고,관절강출혈잉반복발작.결론 획득성응혈인자Ⅹ결핍증림상표현구유이질성,진단의뢰병사화실험실검사,치료포괄공제출혈화치료원발병,예후여환자기출질병상관.
Objective To deepen the understanding of acquired coagulation factor Ⅹ (F Ⅹ) deficiency.Methods The clinical data of 3 patients were analyzed and related literature were reviewed.Results Case 1,a 57-year-old male,secondary to multiple myeloma and amyloidosis,was presented with spontaneous mucous hemorrhage with the level of F Ⅹ ∶ C 1.8％,which kept unchanged after chemotherapy with melphalan,glucocorticoid,and thalidomide,and died of primary disease progression.Case 2,a 41-year-old male with psoriasis,was presented with cerebral and retinal hemorrhage with the level of F Ⅹ ∶ C 26.8％.The signs of hemorrhage were alleviated after the supplement of folic acid,vitamin B12,and vitamin K,and transfusion with red blood cells,platelets,and fresh frozen plasma.Case 3,a 63-year-old female,associated with high level of lupus anticoagulant,was presented with repeated ecchymosis and haemarthrosis with the level of F Ⅹ ∶C 6.1％,which was refractory to prothrombin complex concentrate,methyprednisolone,azathioprine,and rituximab.Conclusions Acquired F Ⅹ deficiency is a rare disorder with variable symptoms.The diagnosis relies on history of disease and laboratory test.Currently,there is no standardized treatment.The prognosis of acquired F Ⅹ deficiency is mainly related to the underlying disease.
