Human coagulation factor Ⅻ （FⅫ）, also called Hageman factor, is a plasma plycoprotein that is functionally deficient in individuals with Hageman trait; which is an inherited trait discovered by chance during preop...Human coagulation factor Ⅻ （FⅫ）, also called Hageman factor, is a plasma plycoprotein that is functionally deficient in individuals with Hageman trait; which is an inherited trait discovered by chance during preoperative blood coagulation screening tests. FⅫ is a single-chain 596-amino-acid zymogen of a serine protease with an approximate molecular weight of 80 000 FⅫ appears to play an important role in blood coagulation,展开更多
BACKGROUND Rheumatoid arthritis(RA)is a common chronic inflammatory autoimmune disease with the main clinical feature of progressive joint synovial inflammation,which can lead to joint deformities as well as disabilit...BACKGROUND Rheumatoid arthritis(RA)is a common chronic inflammatory autoimmune disease with the main clinical feature of progressive joint synovial inflammation,which can lead to joint deformities as well as disability.RA often causes damage to multiple organs and systems within the body,including the blood hemostasis system.Few reports have focused on acquired coagulation dysfunction resulting from vitamin K-dependent coagulation factor deficiency associated with RA.CASE SUMMARY A 64-year-old woman with a history of RA presented to our hospital,complaining of painless gross hematuria for 2 wk.Blood coagulation function tests showed increased prothrombin time,international normalized ratio,and activated partial thromboplastin time.Abnormal blood coagulation factor(F)activity was detected(FII,7.0%;FV,122.0%;and FX,6.0%),indicating vitamin K-dependent coagulation factor deficiency.Thromboelastography and an activated partial thromboplastin time mixed correction experiment also suggested decreased coagulation factor activity.Clinically,the patient was initially diagnosed with hematuria,RA,and vitamin K-dependent coagulation factor deficiency.The patient received daily intravenous administration of vitamin K120 mg,etamsylate 3 g,and vitamin C 3000 mg for 10 d.Concurrently,oral leflunomide tablets and prednisone were administered for treatment of RA.After the treatment,the patient's symptoms improved markedly and she was discharged on day 12.There were no hemorrhagic events during 18 mo of follow-up.CONCLUSION RA can result in vitamin K-dependent coagulation factor deficiency,which leads to acquired coagulation dysfunction.Vitamin K1 supplementation has an obvious effect on coagulation dysfunction under these circumstances.展开更多
BACKGROUND： In adults, vitamin K-dependent coagulation factor deficiency （VKCFD） increases in the recent years. We treated a VKCFD patient with subarachnoid hemorrhage, with favorable outcomes.METHODS： A 19-year-o...BACKGROUND： In adults, vitamin K-dependent coagulation factor deficiency （VKCFD） increases in the recent years. We treated a VKCFD patient with subarachnoid hemorrhage, with favorable outcomes.METHODS： A 19-year-old male student with VKCFD was treated at our hospital. The initial treatment was injection of a large dose of vitamin K and fresh plasma, and then with oral high dose of vitamin K4.RESULTS： At 4 weeks after admission, the focus of hemorrhage subsided, neurological examination was normal, and the patient was discharged.CONCLUSIONS： VKCFD is rare and its diagnosis should be based on the history of the patient and the results of laboratory examinations. A large dose of vitamin K is the fi rst choice of treatment.展开更多
This study examined the changes of activities of vitamin K-dependent clotting factors(VKDCF) under various pathological conditions and explored the relationship between acquired deficiency of VKDCFs and hemorrhage.Cli...This study examined the changes of activities of vitamin K-dependent clotting factors(VKDCF) under various pathological conditions and explored the relationship between acquired deficiency of VKDCFs and hemorrhage.Clinical data of 35 patients who were diagnosed as having acquired deficiency of VKDCF were retrospectively analyzed.Coagulation factors involved in the intrinsic and extrinsic pathways were detected in these patients and 41 control subjects.The results showed that the average activities of VKDCFs were decreased in the patients in comparison to the control subjects and significantly increased after treatment of these patients with vitamin K and blood products.Multivariate regression analysis indicated that decreased activity of VKDCF was not an independent risk factor for bleeding disorders owing to deficiency or metabolic disturbance of vitamin K.It was concluded that acquired deficiency of VKDCF occurs under a variety of pathologic conditions and is closely associated with hemorrhagic events.Administration of vitamin K and transfusion of blood products containing high concentrations of VKDCFs helps alleviate the hemorrhagic diseases.展开更多
BACKGROUND Multiple myeloma patients usually present with CRAB symptoms(hypercalcemia,renal disease,anemia and bone diseases)as initial manifestations.Bleeding symptoms are less common,most of which result from thromb...BACKGROUND Multiple myeloma patients usually present with CRAB symptoms(hypercalcemia,renal disease,anemia and bone diseases)as initial manifestations.Bleeding symptoms are less common,most of which result from thrombocytopenia or infiltration of plasmacytoma.Relatively,coagulopathy is not so common,especially isolated coagulopathy without CRAB manifestations,which is very rare.Herein,we report a 54-year old female who was hospitalized for intermittent and recurrent mild oral mucosal hemorrhage without other bleeding symptoms for almost one month or typical myeloma features.CASE SUMMARY Two months before admission,the patient underwent implantation of a permanent pacemaker due to sick sinus syndrome.Prothrombin time and activated partial thromboplastin time were significantly prolonged.Factor X deficiency was demonstrated to account for the coagulation dysfunction.An M protein peak was shown by serum protein electrophoresis.26.11%of abnormal plasma cells were detected in bone marrow by flow cytometry,expressing CD38,CD138,CD56 and intracellular immunoglobulin Kappa light chain.Bone marrow biopsy also proved the presence of abnormal plasma cells,but Congo red stain was negative.The patient was finally diagnosed with multiple myeloma IgA-κtype.A literature review indicated that factor X deficiency was highly related to amyloidosis.Before bleeding signs,the patient had cardiac arrhythmia,enlargement of the heart,and progressive heart failure;thus,cardiac amyloidosis was suspected.CONCLUSION Bleeding related to coagulation dysfunction is uncommon in multiple myeloma,especially as the initial manifestation.Amyloidosis is a well-recognized cause of isolated acquired factor X deficiency.展开更多
文摘Human coagulation factor Ⅻ （FⅫ）, also called Hageman factor, is a plasma plycoprotein that is functionally deficient in individuals with Hageman trait; which is an inherited trait discovered by chance during preoperative blood coagulation screening tests. FⅫ is a single-chain 596-amino-acid zymogen of a serine protease with an approximate molecular weight of 80 000 FⅫ appears to play an important role in blood coagulation,
基金the National Natural Science Foundation of China,No.81803917 and 81904024.
文摘BACKGROUND Rheumatoid arthritis(RA)is a common chronic inflammatory autoimmune disease with the main clinical feature of progressive joint synovial inflammation,which can lead to joint deformities as well as disability.RA often causes damage to multiple organs and systems within the body,including the blood hemostasis system.Few reports have focused on acquired coagulation dysfunction resulting from vitamin K-dependent coagulation factor deficiency associated with RA.CASE SUMMARY A 64-year-old woman with a history of RA presented to our hospital,complaining of painless gross hematuria for 2 wk.Blood coagulation function tests showed increased prothrombin time,international normalized ratio,and activated partial thromboplastin time.Abnormal blood coagulation factor(F)activity was detected(FII,7.0%;FV,122.0%;and FX,6.0%),indicating vitamin K-dependent coagulation factor deficiency.Thromboelastography and an activated partial thromboplastin time mixed correction experiment also suggested decreased coagulation factor activity.Clinically,the patient was initially diagnosed with hematuria,RA,and vitamin K-dependent coagulation factor deficiency.The patient received daily intravenous administration of vitamin K120 mg,etamsylate 3 g,and vitamin C 3000 mg for 10 d.Concurrently,oral leflunomide tablets and prednisone were administered for treatment of RA.After the treatment,the patient's symptoms improved markedly and she was discharged on day 12.There were no hemorrhagic events during 18 mo of follow-up.CONCLUSION RA can result in vitamin K-dependent coagulation factor deficiency,which leads to acquired coagulation dysfunction.Vitamin K1 supplementation has an obvious effect on coagulation dysfunction under these circumstances.
文摘BACKGROUND： In adults, vitamin K-dependent coagulation factor deficiency （VKCFD） increases in the recent years. We treated a VKCFD patient with subarachnoid hemorrhage, with favorable outcomes.METHODS： A 19-year-old male student with VKCFD was treated at our hospital. The initial treatment was injection of a large dose of vitamin K and fresh plasma, and then with oral high dose of vitamin K4.RESULTS： At 4 weeks after admission, the focus of hemorrhage subsided, neurological examination was normal, and the patient was discharged.CONCLUSIONS： VKCFD is rare and its diagnosis should be based on the history of the patient and the results of laboratory examinations. A large dose of vitamin K is the fi rst choice of treatment.
基金supported by grants from the Ministry of Health of China(No.[2007]353)the National Natural Sciences Foundation of China (N0.30700332)+1 种基金the National Outstanding Youth Foundation(No.30825018)the National Basic Research Program(973 Program,No.2007CB935803)
文摘This study examined the changes of activities of vitamin K-dependent clotting factors(VKDCF) under various pathological conditions and explored the relationship between acquired deficiency of VKDCFs and hemorrhage.Clinical data of 35 patients who were diagnosed as having acquired deficiency of VKDCF were retrospectively analyzed.Coagulation factors involved in the intrinsic and extrinsic pathways were detected in these patients and 41 control subjects.The results showed that the average activities of VKDCFs were decreased in the patients in comparison to the control subjects and significantly increased after treatment of these patients with vitamin K and blood products.Multivariate regression analysis indicated that decreased activity of VKDCF was not an independent risk factor for bleeding disorders owing to deficiency or metabolic disturbance of vitamin K.It was concluded that acquired deficiency of VKDCF occurs under a variety of pathologic conditions and is closely associated with hemorrhagic events.Administration of vitamin K and transfusion of blood products containing high concentrations of VKDCFs helps alleviate the hemorrhagic diseases.
文摘BACKGROUND Multiple myeloma patients usually present with CRAB symptoms(hypercalcemia,renal disease,anemia and bone diseases)as initial manifestations.Bleeding symptoms are less common,most of which result from thrombocytopenia or infiltration of plasmacytoma.Relatively,coagulopathy is not so common,especially isolated coagulopathy without CRAB manifestations,which is very rare.Herein,we report a 54-year old female who was hospitalized for intermittent and recurrent mild oral mucosal hemorrhage without other bleeding symptoms for almost one month or typical myeloma features.CASE SUMMARY Two months before admission,the patient underwent implantation of a permanent pacemaker due to sick sinus syndrome.Prothrombin time and activated partial thromboplastin time were significantly prolonged.Factor X deficiency was demonstrated to account for the coagulation dysfunction.An M protein peak was shown by serum protein electrophoresis.26.11%of abnormal plasma cells were detected in bone marrow by flow cytometry,expressing CD38,CD138,CD56 and intracellular immunoglobulin Kappa light chain.Bone marrow biopsy also proved the presence of abnormal plasma cells,but Congo red stain was negative.The patient was finally diagnosed with multiple myeloma IgA-κtype.A literature review indicated that factor X deficiency was highly related to amyloidosis.Before bleeding signs,the patient had cardiac arrhythmia,enlargement of the heart,and progressive heart failure;thus,cardiac amyloidosis was suspected.CONCLUSION Bleeding related to coagulation dysfunction is uncommon in multiple myeloma,especially as the initial manifestation.Amyloidosis is a well-recognized cause of isolated acquired factor X deficiency.
