RESUMO
Osimertinib, a third-generation epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI), is routinely prescribed as first-line therapy for advanced non-small cell lung cancer, regardless of the presence of the T790M resistance mutation. This study reports a rare case of Factor V inhibitor detection during osimertinib therapy in a patient with lung adenocarcinoma. These findings underscore the importance of vigilant monitoring for coagulation abnormalities during EGFR-TKI therapy.
Assuntos
Acrilamidas , Compostos de Anilina , Carcinoma Pulmonar de Células não Pequenas , Receptores ErbB , Deficiência do Fator V , Neoplasias Pulmonares , Inibidores de Proteínas Quinases , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/genética , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Carcinoma Pulmonar de Células não Pequenas/genética , Carcinoma Pulmonar de Células não Pequenas/patologia , Inibidores de Proteínas Quinases/efeitos adversos , Inibidores de Proteínas Quinases/uso terapêutico , Receptores ErbB/genética , Acrilamidas/uso terapêutico , Acrilamidas/efeitos adversos , Compostos de Anilina/uso terapêutico , Compostos de Anilina/efeitos adversos , Deficiência do Fator V/genética , Masculino , Idoso , Estadiamento de Neoplasias , Mutação , Feminino , Pessoa de Meia-Idade , Adenocarcinoma de Pulmão/tratamento farmacológico , Adenocarcinoma de Pulmão/genética , Adenocarcinoma de Pulmão/patologia , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Indóis , PirimidinasRESUMO
RATIONALE: Coagulation factor V deficiency is rare, and perioperative management of patients with this condition is particularly important, especially during major abdominal surgery. We present a case of a patient with pancreatic duct stones combined with coagulation factor V deficiency. We share our perioperative management experience. PATIENT CONCERNS: A 31-year-old man presented with recurrent upper abdominal pain for 2 years. DIAGNOSES: The diagnosis of pancreatic duct stones in the patient has been established through abdominal computed tomography and magnetic resonance imaging examinations. The diagnosis of factor V deficiency was initially identified through coagulation function tests, revealing significant prolongation of both aPTT and PT. Subsequent testing of coagulation factors and inhibitors demonstrated that the patient has a deficiency in coagulation factor V. Finally, genetic testing revealed that the factor V deficiency in this case is hereditary. INTERVENTIONS: The patient underwent a partial resection of the pancreatic head, and FFP was infused 1 hour before surgery. 600 mL of FFP was instilled 1 hour before the start of surgery along with 10 U of cryoprecipitate. and 600 ml of FFP were added during surgery. Postoperative treatment included intermittent FFP supplemental infusion in the first 5 days after surgery while monitoring the coagulation function. OUTCOMES: The patient underwent a successful surgery without any abnormal bleeding or oozing during the procedure. The postoperative recovery was smooth, with no abnormal bleeding. LESSONS: Patients with a deficiency of coagulation factor V are not contraindicated for surgery. Appropriate Fresh Frozen Plasma (FFP) replacement therapy can ensure the safe conduct of the surgical procedure. For patients with abnormal blood coagulation function, we recommend testing for coagulation factors and inhibitors, as well as performing genetic testing for abnormal coagulation factors, which can provide guidance on marriage and childbirth.
Assuntos
Transtornos da Coagulação Sanguínea , Deficiência do Fator V , Masculino , Humanos , Adulto , Fator V , Coagulação Sanguínea , Tempo de Tromboplastina ParcialRESUMO
Objective: To analyze the gene variation of a genetic coagulation factor â ¤ (Fâ ¤) deficiency pedigree and explore the molecular pathogenesis. Methods: The proband was a 32 years old female. The patient was prone to nose bleeding since childhood which was usually self-healed. On March 10, 2021, the proband went to the First Affiliated Hospital of Air Force Medical University for treatment of knee hematoma caused by a fall. None of the family members reported any history of bleeding. The prothrombin time (PT), activated partial thromboplastin time (APTT) and Fâ ¤ activity (Fâ ¤: C) were detected by clotting method and the Fâ ¤ antigen (Fâ ¤: Ag) was tested with enzyme-linked immunosorbent assay (ELISA). All exons and flanks of F5 gene were determined by Sanger sequencing. Clustalx-2.1-win, PolyPhen-2 and Swiss-PDBViewer software were used to analyze the conservatism of missense variation sites, whether the variations were harmful and their influences on protein structure and function. MutationTaster and NetGene2 software were used to analyze whether the splice site variation was harmful and its effect on the splice site. Results: The PT and APTT of the proband prolonged to 24.0 s and 69.8 s, respectively. The Fâ ¤: C and Fâ ¤: Ag decreased to 6% and 9%, respectively. There were compound heterozygous variations in F5 gene, which included c.911G>A heterozygous missense variation in exon 6 leading to p.Gly276Glu variation and c.5208+1G>A heterozygous missense variation in intron 15. The father and daughter had the p.Gly276Glu heterozygous variation. Her mother and son had the c.5208+1G>A heterozygous variation. Software analysis results of p.Gly276Glu heterozygous variation showed that Gly276 was conserved among homologous species, the variation was harmful, and it could affect the local structure and function of the protein. The c.5208+1G>A heterozygous variation was deleterious and resulted in the disappearance of the splice site, thereby affecting the protein function. Conclusion: The p.Gly276Glu and c.5208+1G>A compound heterozygous variants are deleterious variants associated with the patient's disease and may be the molecular pathogenesis of inherited Fâ ¤ deficiency in this family.
Assuntos
Deficiência do Fator V , Fator V , Humanos , Feminino , Criança , Adulto , Linhagem , Fator V/genética , Mutação , Heterozigoto , Tempo de Tromboplastina Parcial , Deficiência do Fator V/genéticaRESUMO
Aortic regurgitation, a thoracoabdominal aortic aneurysm, chronic myeloid leukemia, and chronic kidney disease were all being treated at two hospitals for an 83-year-old man. He was admitted to the Department of Orthopedics at our hospital with a lumbar compression fracture. Later, he experienced melena, for which the Department of Internal Medicine was consulted. Due to the aberrant results of PT-INR (7.1) and a PTT > 200 seconds on a coagulation test, we suspected the presence of an autoimmune coagulation factor deficiency, and prednisolone immunosuppressive therapy medication was started right away. Due to a sharp decline in FV/5 activity, the presence of FV/5 inhibitors, and the presence of anti-FV/5 autoantibodies, a final diagnosis of autoimmune coagulation factor V (FV/5) deficiency was made. After the start of immunosuppressive therapy, the FV/5 inhibitor and anti-FV/5 autoantibodies disappeared, and the FV/5 activity progressively returned to normal. Disseminated intravascular coagulation-which may have been caused by a known aortic aneurysm-worsened while tapering off prednisolone. Due to the patient's advanced age and other problems, the aneurysm was extensive and inappropriate for surgical repair. The coagulation test findings improved gradually upon initiation of warfarin therapy. Herein, the patient had autoimmune FV/5 deficiency, a rare disorder that made diagnosis and therapy difficult because of the patient's several coexisting conditions.
Assuntos
Coagulação Intravascular Disseminada , Deficiência do Fator V , Masculino , Humanos , Idoso de 80 Anos ou mais , Fator V , Coagulação Intravascular Disseminada/etiologia , Coagulação Intravascular Disseminada/complicações , Prednisolona/uso terapêutico , AutoanticorposRESUMO
Autoimmune factor V deficiency (AiFVD) is a rare bleeding disorder caused by factor V inhibitors. In this report, we present the case of an 89-year-old man who developed bleeding tendency during surgery to create arteriovenous fistula for hemodialysis. The bleeding tendency developed with prolongation of activated partial thromboplastin and prothrombin time, following drug-induced eruption and eosinophilia. Significant reduction in coagulation factor activity and inhibitory pattern in cross-mixing tests suggested the presence of inhibitors to coagulation factors. Subsequently, we detected a factor V inhibitor and anti-factor V autoantibodies was confirmed using enzyme-linked immunosorbent assay with purified human plasma factor V. Thus, the patient was 'definitely diagnosed' with AiFVD in accordance with the diagnostic criteria enacted by the Japanese Ministry of Health, Labor, and Welfare. The bleeding tendency improved after initiating oral prednisolone 50 mg (1 mg/kg) followed by normalization of activated partial thromboplastin time and prothrombin time at the 34th day. After improving the coagulation system prolongation, the inhibitor and autoantibodies has been eradicated. Since it is suggested that drug-induced immune response can cause AiFVD, AiFVD should be considered in patients who undergo hemodialysis and develop failure of hemostasis and drug-induced eruption.
Assuntos
Eosinofilia , Exantema , Deficiência do Fator V , Falência Renal Crônica , Masculino , Humanos , Idoso de 80 Anos ou mais , Testes de Coagulação Sanguínea , Deficiência do Fator V/induzido quimicamente , Deficiência do Fator V/diagnóstico , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Eritema , AutoanticorposRESUMO
Objectives: To report the case of a patient diagnosed with acute mesenteric vein thrombosis (AMVT) associated with Factor V Leiden mutation and a history of in vitro fertilization and embryo transfer and review the literature on risk factors and treatments performed for AMVT. Materials and methods: We reported the case of a 37-year-old pregnant woman. A bibliographic search was carried out in Medline/PubMed and LILACS, filtering by type of language (English and Spanish). Primary cohort studies, cases and controls, case reports and case series were included, which addressed the risk factors associated with the development of acute mesenteric thrombosis during pregnancy and treatments performed. Results: The search identified cases and control studies, case reports and case series related to mesenteric ischemia, pregnancy and in vitro fertilization. The literature reported that the main factors associated with mesenteric ischemia are pregnancy itself, genetic factors, drugs, protein C and protein S deficiency and idiopathic causes. Conclusions: SMV thrombosis is a life-threatening and very rarely seen condition that emerges in pregnancies. The literature suggests that, during gestation, the factors associated with the development of acute mesenteric thrombosis are hypercoagulability induced by pregnancy, the administration of oral estrogen during IVF-ET, and other precipitating factors. More studies are required to better understand the possible additional factors and build better optimal treatment algorithms.
Objetivos: presentar el caso de una paciente diagnosticada con trombosis aguda de la vena mesentérica (TAVM) asociada a mutación de Factor V Leiden y antecedente de fertilización in vitro y transferencia de embriones, y hacer una revisión de la literatura sobre los factores de riesgo y los tratamientos realizados en los casos de TAVM. Materiales y métodos: reporte de un caso de mujer gestante de 37 años. Se realizó una búsqueda bibliográfica en las bases de datos Medline/PubMed y LILACS, filtrando por idioma (inglés y español). Se incluyeron estudios de cohortes primarias, casos y controles, reportes de casos y series de casos que examinaran los factores de riesgo asociados con el desarrollo de trombosis mesentérica aguda durante el embarazo y los tratamientos realizados. Resultados: se identificaron estudios de casos y controles, reportes de casos y series relacionados con isquemia mesentérica, embarazo y fertilización in vitro, y se encontró que los principales factores asociados con isquemia mesentérica son el embarazo mismo, factores genéticos, medicamentos, la deficiencia de proteína C y S, y causas idiopáticas. Conclusiones: la trombosis de la vena mesentérica superior es una condición infrecuente que amenaza la vida y ocurre durante el embarazo. La literatura sugiere que, durante la gestación, los factores asociados con la trombosis mesentérica aguda son la hipercoagulabilidad inducida por el embarazo, la administración de estrógeno oral durante el proceso de fertilización in vitro y transferencia de embriones, y otros factores desencadenantes. Es necesario realizar más estudios para comprender mejor los posibles factores adicionales y desarrollar mejores algoritmos para un tratamiento óptimo.
Assuntos
Humanos , Feminino , Gravidez , Adulto , Pessoa de Meia-Idade , Trombose , Deficiência do Fator V , Gravidez , Fertilização in vitro , Estudos de Casos e Controles , Gestantes , Veias MesentéricasRESUMO
BACKGROUND: Factor V deficiency is a rare disease, with an incidence of one in a million. Symptoms are mostly scant, and it is often diagnosed by the presence of an abnormality on PT-INR or APTT. In addition, no established therapy exists and platelet dysfunction is seldom found to be concomitant with this disease CASE PRESENTATION: A 64-year-old man who had both factor V deficiency and platelet dysfunction had angina in the past year. Coronary surgery was required, and we successfully performed coronary artery bypass grafting under strategic planned platelet transfusion with additional adequate cryoprecipitates transfusion. No perioperative problems nor any postoperative major bleeding issues were observed. The postoperative course was also uneventful. CONCLUSION: Strategic planned platelet transfusion with the additional transfusion of an adequate amount of cryoprecipitates is thus considered to be feasible for cases presenting with factor V deficiency and platelet dysfunction.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Deficiência do Fator V , Masculino , Humanos , Pessoa de Meia-Idade , Deficiência do Fator V/terapia , Ponte de Artéria Coronária , Transfusão de Sangue , Hemorragia Pós-Operatória , Transfusão de PlaquetasRESUMO
Acquired factor V deficiency is a rare disease that presents with various bleeding symptoms because of the acquired production of factor V inhibitors and decrease in factor V activity. We have experienced five cases of acquired factor V deficiency diagnosed on the basis of abnormalities in coagulation tests in the last 10 years. All five patients were older men, of whom one had no bleeding symptoms, and three had a history of renal failure and malignant tumors. In the cross-mixing test, two of three cases demonstrated an inhibitor pattern, but one case showed a deficient pattern. In all cases, steroid treatment improved factor V activity as well as prothrombin time and activated partial thromboplastin time. However, patients with intracranial hemorrhage had a poor prognosis. Although this disease is rare, careful management is necessary, especially in the absence of bleeding symptoms and where cross-mixing test does not show an inhibitor pattern.
Assuntos
Deficiência do Fator V , Idoso , Testes de Coagulação Sanguínea/efeitos adversos , Fator V/genética , Deficiência do Fator V/complicações , Deficiência do Fator V/diagnóstico , Hemorragia/etiologia , Humanos , Masculino , Tempo de Tromboplastina Parcial , Tempo de ProtrombinaRESUMO
Congenital factor V deficiency is an extremely rare abnormality and may be associated with significant bleeding as a result of trauma or surgery. Perioperative management primarily includes the administration of fresh frozen plasma to replace the deficient clotting factor. Acute administration of multiple blood products is a risk factor for transfusion-associated circulatory overload. Herein, the case of a 71-year-old man with an ejection fraction of 13% and a history of congenital factor V deficiency who was at risk for significant bleeding and transfusion-associated circulatory overload who underwent successful complex extraction of a right atrial pacing lead is reported.
Assuntos
Deficiência do Fator V , Reação Transfusional , Idoso , Fatores de Coagulação Sanguínea , Hemorragia , Humanos , Lasers , MasculinoRESUMO
OBJECTIVE: The aim was to investigate the clinical characteristics and molecular pathogenic mechanism of twins with congenital factor V (FV) deficiency. METHODS: We comprehensively analyzed the clinical manifestations and laboratory test results of a set of twins and their parents and performed point mutation analysis with direct high-throughput exon sequencing. RESULTS: The prothrombin time and activated partial thromboplastin time were prolonged for both probands, and the FV activity levels were 13.0% and 9.8%. Next-generation sequencing showed that the affected individuals harbored a paternal c.5113A>C (p.S1705R) and a maternal c.4949C>T (p.A1650V) heterozygous variants in the FV gene, which conformed to an autosomal recessive inheritance pattern. This is the first report of these point mutations. The older boy also had a congenital patent foramen ovale. CONCLUSION: In this set of twins, missense mutations of the FV gene were related to congenital FV deficiency but unrelated to the patent foramen ovale observed in the older boy.
Assuntos
Proteínas de Ligação a Calmodulina/genética , Deficiência do Fator V , Forame Oval Patente , Proteínas dos Microfilamentos/genética , Resistência à Proteína C Ativada , Fator V/genética , Deficiência do Fator V/congênito , Deficiência do Fator V/genética , Heterozigoto , Humanos , Mutação , Linhagem , FenótipoRESUMO
Deficiency of factor V is a congenital autosomal recessive coagulopathy associated with mutations in the F5 gene that results in mild-to-severe bleeding episodes. Factor V is a component of the prothrombinase complex responsible for accelerating conversion of prothrombin to thrombin. At the present time there are no therapeutic factor V concentrates available. This study was designed to lay the preliminary foundations for future cell-based therapy for patients with severe factor V deficiency. The study showed that hepatospheres, which produce coagulation factors VIII, IX, and V, synthetize and store intracellular glycogen and express albumin levels up to 8 times higher than those of undifferentiated cells. Factor IX and factor V gene expression increased significantly in hepatospheres as compared to undifferentiated cells, whereas factor VIII gene expression remained constant. The factor V protein was detected in the hepatospheres´ secretome. Considering the enormous potential of mesenchymal stem cells as therapeutic agents, this study proposes a highly reproducible method to induce differentiation of mesenchymal stem cells from human placenta to factor V-producing hepatospheres. This strategy constitutes a preliminary step towards a curative treatment of factor V deficiency through advanced therapies such as cell therapy.
Assuntos
Terapia Baseada em Transplante de Células e Tecidos/métodos , Decídua/citologia , Deficiência do Fator V/terapia , Células-Tronco Mesenquimais/citologia , Células-Tronco Mesenquimais/metabolismo , Albuminas/genética , Albuminas/metabolismo , Técnicas de Cultura de Células , Diferenciação Celular , Fator IX/genética , Fator IX/metabolismo , Fator V/genética , Fator V/metabolismo , Fator VIII/genética , Fator VIII/metabolismo , Feminino , Hepatócitos/citologia , Hepatócitos/metabolismo , Humanos , Secretoma/metabolismo , Esferoides Celulares/citologia , Esferoides Celulares/metabolismoRESUMO
Objective: To explore the molecular pathogenesis of a family with hereditary factor â ¤ (Fâ ¤) deficiency. Methods: All the exons, flanking sequences, 5' and 3' untranslated regions of the F5 of the proband, and the corresponding mutation sites of the family members were analyzed via direct DNA sequencing. The CAT measurement was used to detect the amount of thrombin produced. The ClustalX software was used to analyze the conservation of mutation sites. The online bioinformatics software, Mutation Taster, PolyPhen-2, PROVEAN, LRT, and SIFT were applied to predict the effects of mutation sites on protein function. The Swiss-PdbViewer software was used to analyze the changes in the protein model and intermolecular force before and after amino acid variation. Results: The proband had a heterozygous missense mutation c.1258G>T (p.Gly392Cys) in exon 8 of the F5, and a heterozygous deletion mutation c.4797delG (p.Glu1572Lys fsX19) in exon 14, which results in a frameshift and produces a truncated protein. Her grandfather and father had p.Gly392Cys heterozygous variation, whereas her maternal grandmother, mother, little aunt, and cousin all had p.Glu1572LysfsX19 heterozygous variation. The ratio of proband's thrombin generation delay to peak time was significantly increased. Conservation analysis results showed that p.Gly392 was located in a conserved region among the 10 homologous species. Five online bioinformatics software predicted that p.Gly392Cys was pathogenic, and Mutation Taster also predicted p.Glu1572Lys fsX19 as a pathogenic variant. Protein model analysis showed that the replacement of Gly392 by Cys392 can lead to the extension of the original hydrogen bond and the formation of a new steric hindrance, which affected the stability of the protein structure. Conclusion: The c.1258G>T heterozygous missense mutation in exon 8 and the c.4797delG heterozygous deletion mutation in exon 14 of the F5 may be responsible for the decrease of Fâ ¤ levels in this family.
Assuntos
Deficiência do Fator V , Éxons , Deficiência do Fator V/genética , Feminino , Heterozigoto , Humanos , Mutação , LinhagemRESUMO
Factor V deficiency is an extremely rare hematologic disorder with an incidence of one in one million. However, the risks related to cardiac surgery employing cardiopulmonary bypass in patients with factor V deficiency are not well established. Herein, we report the case of a 71-year-old male who was incidentally diagnosed with acquired factor V deficiency underwent mitral valve repair for severe mitral regurgitation. The patient was treated preoperatively with an adrenocorticosteroid immunosuppressant therapy; the procedure was performed safely with a positive outcome.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Deficiência do Fator V , Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Mitral , Idoso , Ponte Cardiopulmonar , Deficiência do Fator V/diagnóstico , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Masculino , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/cirurgia , Resultado do TratamentoRESUMO
Since acquired factor V inhibitor (FV-INH) has been first reported in Germany in 1955, about 200 cases have been recorded globally. The incidence of FV-INH is extremely low, with a rate of 0.023-0.09 per million persons per year. FV-INH formation is caused by infection, use of antibiotics and other drugs, surgery, and diseases, including malignancy and autoimmune disorder. Some patients with FV-INH present with abnormal clinical laboratory test results but have no hemorrhagic symptoms. Others experience life-threatening bleeding. Moreover, thrombosis can sometimes occur. The diagnosis is based on prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT), an inhibitor pattern shown by a cross-mixing test of PT and APTT, decreased factor V activity, and detection of FV-INH. Treatment includes hemostatic and immunosuppressive therapy. However, in some cases, the monitoring of progression alone is appropriate. In terms of hemostatic therapies, infusion of platelet concentrates and administration of recombinant factor VIIa are highly useful. However, no definitive treatment strategy has been established. In about 50% of cases, FV-INH is eliminated spontaneously. Therefore, immunosuppressive therapy is recommended only for hemorrhagic patients or those at high risk of hemorrhage. Prednisolone is generally used for the management of immunosuppression. However, some reports have shown that the administration of rituximab, cyclophosphamide, and intravenous immunoglobulin and plasma-exchange can be utilized as treatments.
Assuntos
Fator V/antagonistas & inibidores , Testes de Coagulação Sanguínea , Deficiência do Fator V , Hemorragia , Humanos , Tempo de Tromboplastina ParcialRESUMO
INTRODUCTION: Congenital factor V deficiency (FVD) is a rare bleeding disorder characterized by low or undetectable plasma factor V (FV) levels leading to mild to severe bleeding symptoms. Currently, more than 100 mutations have been reported in F5. We herein report a patient with FVD from mutations in the F5 gene. PATIENT CONCERNS: A 52-year-old man with prolonged prothrombin time and activated partial thromboplastin time corrected by mixing test on preoperative screening. His past medical or family history was not remarkable. DIAGNOSIS: Factor assays revealed a markedly reduced FV activity at 7%. Other factors were not decreased. DNA sequencing analysis to detect F5 gene mutations showed the patient was compound heterozygous for c.286G>C (p.Asp96His) and c.2426del (p.Pro809Hisfs*2). Asp96His was previously described missense mutation and Pro809Hisfs*2 was a novel deleterious mutation. INTERVENTIONS: Fresh-frozen plasma was administered to supplement FV before surgery. OUTCOMES: Subsequent factor assays revealed temporarily increased FV activity at 33%. CONCLUSION: As was the case in our patient, genotype-phenotype correlations are poor in FVD, and molecular genetic test is necessary to confirm the diagnosis.
Assuntos
Deficiência do Fator V/genética , Fator V/genética , Mutação , Diagnóstico Diferencial , Deficiência do Fator V/cirurgia , Heterozigoto , Humanos , Masculino , Pessoa de Meia-Idade , FenótipoRESUMO
BACKGROUND: Severe factor V deficiency is an extremely rare coagulation disorder. Patients with factor V activity <5% usually become symptomatic in early childhood. CASE DESCRIPTION: We report the case of an 82-year-old woman with incidentally diagnosed severe factor V deficiency, who developed a symptomatic chronic subdural hematoma, requiring burr hole craniostomy. Successful management was achieved by a multidisciplinary approach. Preoperatively, factor V activity was increased from 2% to 50% by administration of 25 mL/kg body weight of fresh frozen plasma over 30 minutes under close cardiopulmonary monitoring in the intensive care unit. Straight afterward, the patient was transferred to the operating room where surgery was performed under general anesthesia. Burr hole craniostomy could be performed without perioperative complications. In the postoperative days, there was no relevant recurrence of the subdural hematoma in the follow-up computed tomography scans under frequent control of coagulation parameters. However, despite further transfusion of fresh frozen plasma, factor V activity did not increase >16%. The patient was discharged without any neurologic deficits. In a hemostaseologic follow-up 2 months after surgery, factor V activity <1% was confirmed with evidence of a factor V inhibitor in the modified Bethesda assay. Most likely, the patient suffered from an acquired form of factor V deficiency with preformed antibodies that had been boosted by the initial treatment with fresh frozen plasma. CONCLUSIONS: We conclude that in this rare bleeding disorder, intracranial surgery was successfully managed because of a thoroughly planned perioperative therapeutic strategy. However, if there is time prior to surgery, a full checkup of the bleeding disorder is advisable.
Assuntos
Deficiência do Fator V/diagnóstico por imagem , Deficiência do Fator V/cirurgia , Hematoma Subdural Crônico/diagnóstico por imagem , Hematoma Subdural Crônico/cirurgia , Assistência Perioperatória/métodos , Índice de Gravidade de Doença , Idoso de 80 Anos ou mais , Gerenciamento Clínico , Deficiência do Fator V/complicações , Feminino , Hematoma Subdural Crônico/complicações , Humanos , Resultado do TratamentoRESUMO
Acquired factor V (FV) inhibitor is a rare disorder. Herein we report a case of an 82-year-old Japanese woman with FV inhibitor exhibiting a pseudo decline in the activities of the multiple coagulation factors. After rectal cancer surgery, she received antibiotic therapy for wound infection. As prothrombin and activated partial thromboplastin time was prolonged, heparin for atrial fibrillation was discontinued without improvement. Coagulation factor activity assays revealed deficiencies in II, V, VII, VIII, IX, X, XI, and XII factor activities; in particular, the FV activity was markedly decreased to <1%. The cross-mixing test findings revealed an inhibitor pattern, and multiple coagulation factor inhibitors were positive. The FV inhibitor level was high at 62 Bethesda U/ml. The patient exhibited no bleeding tendency with the prolonged wound infection without immunosuppressive therapy. The inhibitor disappeared four months after the onset.
Assuntos
Antibacterianos/efeitos adversos , Deficiência do Fator V/induzido quimicamente , Infecção da Ferida Cirúrgica/tratamento farmacológico , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Testes de Coagulação Sanguínea , Feminino , Humanos , Tempo de Tromboplastina ParcialRESUMO
PURPOSE: The aim of the present study was to analyze the management of single dental extractions and postoperative bleeding in patients with a diagnosis of factor V deficiency. A careful evaluation of each case will allow the team to categorize the risk and operate safely, minimizing the incidence of intraoperative and postoperative complications. If necessary, the oral-maxillofacial surgeon can choose to do so in collaboration with the hematologist on a case-by-case basis. PATIENTS AND METHODS: The present retrospective study included 5 patients with mild congenital factor V deficiency who had undergone at least 1 dental extraction. Mouth rinse with tranexamic acid, nonresorbable sutures, and gelatin sponge packed in the alveolar socket were used to obtain hemostasis. No systemic therapies, such as fresh frozen plasma, platelet concentrate, or recombinant activated factor VII, were administered. RESULTS: Twenty-five teeth were extracted. The factor V plasma levels ranged from 14.1 to 22.4%. Local antihemorrhagic treatments resulted in good hemostasis. No hemorrhagic complications or intraoperative or postoperative major bleeding was observed. CONCLUSIONS: Dental extractions appear to be safe procedures for patients with mild factor V deficiency when a bleeding risk assessment has been performed in conjunction with a hematologist and an appropriate treatment protocol is followed. Our treatment protocol was found to be effective and well tolerated by all the patients.