Spontaneous Hemorrhages Caused by Plasma-Thromboplastin-Antecedent Deficiency

Henry, E

doi:10.1001/jama.1956.72970250001008

Cited by 21 publications

(7 citation statements)

References 2 publications

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“…In contrast to severe haemophilia A and B, spontaneous bleeding is not usually a feature, but can occur. Cases have been reported of individuals who suffered a massive haemothorax [13], a cerebral haemorrhage [24], a subarachnoid haemorrhage [25], and a spinal epidural haematoma with Brown–Sequard syndrome [26]. Spontaneous haemarthroses are rare but have been reported, even in individuals with partial deficiency [27].…”

Section: Clinical Picture and Inheritancementioning

confidence: 99%

Factor XI deficiency and its management

Bolton‐Maggs

2000

Haemophilia

110

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Factor XI deficiency has a more variable bleeding tendency than haemophilia A or B. Individuals with severe deficiency have only a mild bleeding tendency, which is typically provoked by surgery, but the risk of bleeding is not restricted to individuals with severe deficiency. The bleeding tendency varies between individuals with similar factor XI levels, and sometimes the bleeding tendency of an individual may vary. The reasons for this are not fully understood, although in cases of severe deficiency there is some correlation between phenotype and genotype. Factor XI is activated by thrombin. The role of factor XI in physiological processes has become clearer since this fact was discovered, and the discovery has contributed to a revised model of blood coagulation. Factor XI deficiency occurs in all racial groups, but is particularly common in Ashkenazi Jews. The factor XI gene is 23 kilobases long. Two mutations are responsible for most factor XI deficiency in the Ashkenazi population, but a number of other mutations have now been reported in other racial groups. Individuals with factor XI deficiency may need specific therapy for surgery, accidents, and dental extractions. Several therapies are available which include fresh frozen plasma, factor XI concentrates, fibrin glue, antifibrinolytic drugs, and desmopressin. Each has advantages and risks to be considered. Factor XI concentrate may be indicated for procedures with a significant risk of bleeding especially in younger patients with severe deficiency, but its use in older patients has been associated with thrombotic phenomena. If fresh frozen plasma is to be used it is preferable to obtain one of the virally inactivated products. Fibrin glue is a useful treatment which deserves further study.

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Section: Clinical Picture and Inheritancementioning

confidence: 99%

Factor XI deficiency and its management

Bolton‐Maggs

2000

Haemophilia

110

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“…These authors also report death from bleeding in childbirth of the mother of another FXI deficient woman. Another woman of 38, who bled after delivery, had a spontaneous intracranial bleed, and also suffered from heavy periods, was eventually treated with monthly plasma infusions 7 days prior to her period to try to control her excessive blood loss [ 6]. It has been noted in many series that bleeding is not predictable in an individual, or between individuals in the same kindred, and is not well related to the FXI level in partial deficiency [ 3, 7].…”

Section: Fxi Deficiency and Menorrhagiamentioning

confidence: 99%

Bleeding problems in factor XI deficient women

Bolton-Maggs

1999

Haemophilia

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Factor XI deficiency affects both sexes. Some bleeding problems are specific to women, and have been overlooked in the past. Women with factor XI deficiency are prone to menorrhagia and to bleeding complications after childbirth. These may occur in women with partial deficiency, as well as in those with severe deficiency. Instrumental delivery and gynaecological surgery need careful planning in such women with close collaboration between the obstetrician and haematologist.

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“…Patients with factor XI deficiency need specific management during surgery. Previously, adverse surgical outcomes of patients with factor XI deficiency have been reported, including cerebral hemorrhage [ 6 ] and spinal epidural hematoma [ 7 ]. Different surgical management strategies, each with varying degrees of risk, have been reported, including FFP, factor XI concentrates, and desmopressin [ 8 ].…”

Section: Case Presentationmentioning

confidence: 99%

Anesthetic management using peripheral nerve block in patients with factor XI deficiency: a case report

et al. 2016

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Factor XI deficiency is rare but may cause life-threatening bleeding during the perioperative period. The clinical manifestation of factor XI deficiency is characterized by bleeding tendency. This unpredictable bleeding tendency makes anesthetic management difficult. We report a case of a partial duodenectomy in a patient with factor XI deficiency. The patient was scheduled for duodenectomy because of a duodenal tumor. When checked for coagulation before surgery, the patient was found to have a remarkably prolonged activated partial thrombin time, and further investigation revealed factor XI deficiency. Fresh frozen plasma was transfused before surgery, and general anesthesia and peripheral nerve block were performed. In the present case of factor XI deficiency, supplementation with clotting factor and proper anesthetic management were important to prevent severe complications.

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Spontaneous Hemorrhages Caused by Plasma-Thromboplastin-Antecedent Deficiency

Cited by 21 publications

References 2 publications

Factor XI deficiency and its management

Factor XI deficiency and its management

Bleeding problems in factor XI deficient women

Anesthetic management using peripheral nerve block in patients with factor XI deficiency: a case report

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