Rare Bleeding Disorders

Peyvandi, Flora; Menegatti, Marzia

doi:10.1002/9781444323160.ch42

Cited by 13 publications

(14 citation statements)

References 87 publications

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“…Bleeding tendency in factor XI deficiency do not correlate with the factor XI level, therefore the optimal treatment should be determined by the clinical course and individual circumstances [4][5]. A retrospective analysis of bleeding complications in patients with factor XI deficiency revealed that extent and severity of the surgery influences the risk of bleeding.…”

Section: Discussionmentioning

confidence: 99%

Successful laparoscopic radical prostatectomy in a patient with factor XI deficiency

Murányi¹,

Benyó²,

Kiss³

et al. 2016

IJCRI

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International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. Factor XI deficiency is a rare type of hemophilia. It is associated with profuse bleeding after traumas and surgical procedures. In a patient with factor XI deficiency, the serum coagulation factor level does not predict bleeding tendency appropriately. IJCRI publishesOperations in sites without fibrinolytic activity are less likely to be complicated by bleeding, while surgery in areas with fibrinolytic activity, like prostatectomy, increases the risk of bleeding. The optimal treatment should be determined by individual circumstances. Radical prostatectomy is a procedure when severe bleeding requiring transfusion can occur even without any bleeding disorder. At the same time radical prostatectomy carries a significant risk of potentially fatal thromboembolism. Thus keeping the balance between procoagulant and anticoagulant activity is the challenging part in cases of radical prostatecomy of factor XI deficiency. Case Report: A 67-year-old patient is presented with organ confined intermediate-risk prostate cancer. Prolonged bleeding after tooth extraction and prostate biopsy indicated assessment of hemostatic system. Laboratory tests revealed factor XI deficiency. Successful laparoscopic extraperitoneal radical prostatectomy and bilateral pelvic lymphadenectomy was performed with low amount of blood loss. Recombinant activated coagulation factor VII was administered to prevent perioperative bleeding. The postoperative period was uneventful, no complications were observed. Conclusion: Despite the high risk of hemorrhage, laparoscopic radical prostatectomy is a feasible procedure even in a patient with factor XI deficiency, providing that the patient receive adequate preparation and a meticulously performed operation carried out by an experienced surgeon.

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Section: Discussionmentioning

confidence: 99%

Successful laparoscopic radical prostatectomy in a patient with factor XI deficiency

Murányi¹,

Benyó²,

Kiss³

et al. 2016

IJCRI

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“…Keywords: hemophilia A, bleeding, factor 8 giriŞ Hemofili A; faktör 8 (F8) eksikliğine bağlı, X'e bağlı resesif geçişli, en sık rastlanan kalıtsal kanama bozukluğudur (1) . F8 eksikliği; yaralanma, diş çekimi ve cerrahi işlemler sonrası uzamış kanamalara ve gecikmiş yara yeri iyileşmesine neden olur (2) .…”

Section: öZunclassified

The anesthetic approach for thyroid medullary carcinoma patient with severe hemophilia a diagnosis: Case report

Demirgan¹,

Kutluyurdu²,

Şitilci³

et al. 2015

IKSST

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ÖZ Anahtar kelimeler: hemofili A, kanama, faktör 8 ABSTrACT Hemophilia A is the most frequently observed genetic bleeding disorder with an X-linked recessive trait resulting from the deficiency of factor 8 (F8). F8 deficiency leads to prolonged bleeding episodes after traumas, tooth extraction, and surgical procedures. Surgical procedures in hemophilia A patients present peculiarities. The aim of this study is to discuss principles of preoperative preparation

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“…The incidence of this deficiency is higher in countries with high rates of consanguineous marriages [3]. In Pakistan, close consanguineous unions continue to be extremely common as in South West Asia.…”

Section: Introductionmentioning

confidence: 99%

“…Patients usually present at early neonatal period with prolonged umbilical stump bleeding, post circumcision bleeding, bruises, mucosal hemorrhage, cerebral bleeding, hemarthrosis, hematoma, intracranial hemorrhage, epistaxis, menorrhagia and joint bleeding. However, life-threatening bleeding such as central nervous and musculoskeletal bleeding appears to be less frequent than in hemophilia [6].…”

Section: Introductionmentioning

confidence: 99%