Anaesthesia Management of a Patient with Factor XI Deficiency

Adibelli, Bilgehan; Araz, Çoşkun; Ersoy, Zeynep; Kayhan, Zeynep

doi:10.5152/tjar.2016.90267

Cited by 4 publications

(11 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…FXI deficiency, also known as TCH, is a rare disease linked to chromosome 4,[ 6 ] with variable inheritance and a 1/1,000,000 inhabitants incidence,[ 6 7 ] reaching 1/450 people in Ashkenazi population. [ 7 ] Its defect causes an intrinsic coagulation pathway alteration that should be suspected when an isolated prolongation of APTT is present with normal prothrombin time.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Hemophilia C management in obstetric anesthesia

et al. 2022

View full text Add to dashboard Cite

Coagulation alterations might represent a problem in obstetric anesthesia considering that they may contraindicate neuraxial techniques and worsen a case of uterine atony with more severe bleeding if they are not correctly recognized and treated. We report the case of a parturient diagnosed with severe factor XI deficiency during the delivery progress. In this case, non-steroidal anti-inflammatory drugs and neuraxial techniques were avoided and intravenous patient-controlled analgesia with boluses of remifentanil was used for pain management. Treatment with tranexamic acid and fresh frozen plasma was initiated and the absence of urgent factor XI availability was notified. Due to no progression of labor, cesarean section was required and a general anesthesia was performed. During the procedure, uterine atony occurred. Uterine massage and several uterotonic drugs were needed to control it. The patient remained stable and the delivery was accomplished without further incidents. The objective of this report is to present the pain, coagulation and bleeding management of a patient with hemophilia C in our obstetric department and to alert for the need of multidisciplinary work to successfully approach this type of patient.

show abstract

Section: Discussionmentioning

confidence: 99%

“…[ 7 ] Its defect causes an intrinsic coagulation pathway alteration that should be suspected when an isolated prolongation of APTT is present with normal prothrombin time. [ 6 8 ]…”

Section: Discussionmentioning

confidence: 99%

Hemophilia C management in obstetric anesthesia

et al. 2022

View full text Add to dashboard Cite

show abstract

“…In addition to bleeding history and factor levels, the type of procedure (major or minor) as well as site of surgery should be taken into consideration during preprocedural planning, as sites such as pharynx and urinary tract has higher fibrinolytic activity and are more prone to bleed [3]. For major procedures in individuals with severe factor XI deficiency or significant bleeding history, monitoring of factor XI activity in the perioperative period with replenishment using factor XI concentrates or FFP is recommended [14][15][16][17][18][19][20][21]. Two plasma-derived factor XI concentrates namely Hemoleven (a high purity product from LFB Biomedicaments, Les Ulis, France) and factor XI concentrate (a partially purified product from Bioproducts Laboratory, Elstree, UK) have shown efficacy, but have yet to be widely used due to unavailability in many parts of the world including USA.…”

Section: Discussionmentioning

confidence: 99%

“…Plasma exchange in these cases can help raise factor XI level while removing any potential non-neutralizing inhibitor in an isovolumetric fashion [24]. With careful attention to these factors, major surgeries can be performed in these patients [14][15][16][17][18][19][20][21]. The same principles also apply in the management of patients with bleeding complications such as subdural hemorrhage [19].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Hemophilia C: A Case Report With Updates on Diagnosis and Management of a Rare Bleeding Disorder

Jayakrishnan¹,

Shah²,

Mewawalla³

2019

J Hematol

View full text Add to dashboard Cite

Hemophilia C or factor XI deficiency is a rare clotting disorder with prevalence of only 1 per 1 million. A 24-year-old male with multiple abdominal surgeries complicated by wound infections and poor healing was admitted to plastic surgery service for an elective abdominoplasty. Hematology was consulted for increased intraoperative and postoperative bleeding. Laboratory workup showed high-normal activated plasma thromboplastin time of 31 s (reference: 23-34 s), prothrombin time (PT) of 15 s (reference: 11.8-14.3 s) and internationalized normal ratio (INR) of 1.2. Patient had normal factors VIII, IX, XIII levels and normal von Willebrand's factor level. The factor XI level came back at 0.28 (0.44-1.43 U/mL) diagnostic for intermediate factor XI deficiency. Factor XI is responsible for thrombin generation after clotting is initiated as well as clot stabilization. The confirmatory test is factor XI assay. The management of factor-XI deficiency is based on history of bleeding and nature of the procedure.

show abstract

Factor XI Deficiency in a Patient with Cervical Spondylotic Myelopathy

Serikyaku

Higa

Yara

et al. 2020

Spine

View full text Add to dashboard Cite

Study Design.Case report.Objective.To summarize the clinical manifestations and treatment of Factor XI deficiency in a patient with cervical spondylotic myelopathy.Summary of Background Data.Factor XI deficiency is a rare genetic bleeding disorder caused by reduced levels and insufficient activity of a coagulation factor XI. It is claimed to be associated with prominent bleeding in case of trauma and surgery irrelevant to the FXI level. This is the first ever case of a patient with factor XI deficiency with cervical spondylotic myelopathy.Methods.A case was investigated retrospectively and the relevant literature was reviewed.Results.A 66-year-old man with a 2-months history of lack of finger dexterity and gait disturbance was referred to our department. He did not have a history of bleeding or coagulation disorder nor did his family. Magnetic resonance imaging (MRI) of the cervical spine revealed spinal canal stenosis at C3/4 to C5/6 and intramedullary hyperintensity at C3/4 on the :T2 weighted image (T2WI). Preoperative examination revealed no abnormal findings but a severe prolonged activated partial-thromboplastin time (APTT) of 139.8 seconds. Coagulation factor activity assay revealed severe deficiency of factor XI (<0.1%). In accordance with hematologist's recommendation, four units of fresh frozen plasma (FFP) were transfused on the day before surgery and APTT assayed early morning on the day of surgery was 70.5 seconds. An additional four units of FFP were transfused during the surgery and APTT was 60 seconds. The postoperative course was uneventful and the patient was discharged on the postoperative day 14.Conclusion.Factor XI deficiency patients may develop excessive bleeding after trauma or surgery. Preoperative examination with prolonged APTT should be pursued until a diagnosis of is made. Under diagnosis of Factor XI deficiency, meticulous attentions are required for perioperative bleeding management including postoperative hematoma in spinal surgery.Level of Evidence:5

show abstract

Anaesthesia Management of a Patient with Factor XI Deficiency

Cited by 4 publications

References 9 publications

Hemophilia C management in obstetric anesthesia

Hemophilia C management in obstetric anesthesia

Hemophilia C: A Case Report With Updates on Diagnosis and Management of a Rare Bleeding Disorder

Factor XI Deficiency in a Patient with Cervical Spondylotic Myelopathy

Contact Info

Product

Resources

About