Surgical Procedures in Unsuspected Hemophilia

Coy., James Hardie; Bivins, Brack A.; Belin, Robert P.

doi:10.1001/archsurg.1974.01360060101028

Cited by 6 publications

(2 citation statements)

References 4 publications

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“…Mild hemophilia is defined by factor levels between 5% and 40%. While individuals with the moderate (1–5%) and severe (<1%) forms of hemophilia generally have a higher risk of spontaneous and life‐threatening bleeds, the bleeding associated with mild hemophilia A is usually reported as episodic, occurring during surgery or following trauma [1–3]. Mild hemophilia may also be characterized by joint and muscle hemorrhage, leading over time to chronic pain and hemoarthropathy with eventual joint replacement surgeries [4].…”

Section: Introductionmentioning

confidence: 99%

Health‐related quality of life in a cohort of adult patients with mild hemophilia A

Walsh

Macgregor

Stuckless

et al. 2008

Journal of Thrombosis and Haemostasis

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Summary. Objectives: To compare the health-related quality of life among adult males affected with mild hemophilia A due to the same mutation (Val2016ala) to that of unaffected age and sex matched controls from the same general population. Methods: The Short-Form 36 (SF-36) and Health Assessment Questionnaire (HAQ) were used to measure health-related quality of life and physical function. Other measures included bleeding history, a measure of joint damage, body mass index, age, and viral infection status. Cross-sectional data were collected through research clinics and a retrospective chart audit over a two-year period. Results and Conclusions: The study included 47 affected males and 33 controls. The affected males had a higher level of co-morbidity, prior bleeding, and existing joint damage than controls. With the exception of the social function and health transition scales, mean scores for each of the SF-36 domains were worse among affected males. Mean differences were more than a clinically important five points in five of eight domains, with the general health scale showing more than a 10-point difference. Despite the degree of difference noted, only two of the differences were statistically significant (general health and role emotional scales) because of the small sample size and considerable individual variation in SF-36 scale scores. Multiple regression analyses suggested existing joint damage and presence of heart disease as the strongest associates of lower physical healthrelated quality of life. Joint damage in turn was partly related to prior hemarthroses. Compared to the Canadian population, affected males had lower scores in six out of eight SF-36 domains as well as the physical component summary score. There were no significant differences found in the HAQ scores between the two groups. So-called mild hemophilia A was associated with a negative effect on physical health-related quality of life, contributed to by joint damage as a result of prior bleeding.

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Section: Introductionmentioning

confidence: 99%

Health‐related quality of life in a cohort of adult patients with mild hemophilia A

Walsh

Macgregor

Stuckless

et al. 2008

Journal of Thrombosis and Haemostasis

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“…In such case, following disorders should be considered, such as multiple transfusions of bank blood, intravascular coagulation, incompatible transfusion, undetected anticoagulant therapy, platelet deficiency, shock with anoxia, and unsuspected mild hemophilia. 16) To detect the cause of intraoperative unexpected hemorrhagic diathesis, we should immediately confirm medications of the patient, coagulation function, and blood type of transfusion. Adequate transfusion should be initiated if necessary.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Giant Solid Hemangioblastoma Accompanied with Hemophilia Type A

Ujihara

Maki

Chin

et al. 2018

NMC Case Report Journal

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The surgical removal of giant solid hemangioblastoma involves a high risk of perioperative bleeding and requires attentive hemostasis. Here, we present a case of a giant solid hemangioblastoma accompanied with hemophilia which was previously undiagnosed. A 35-year-old man without any past medical history was admitted with diplopia and ocular motility disorder. computed tomography (CT) and magnetic resonance imaging (MRI) revealed obstructive hydrocephalus and a solid giant tumor of more than 4.0 cm in diameter in the right cerebellopontine angle (CPA). Hemangioblastoma was suspected on cerebral angiography. After ventriculoperitoneal shunt for obstructive hydrocephalus, oozing from the skin incision continued for several days. Hemophilia type A was diagnosed based on the result of laboratory blood coagulability examination. Supplemental administration of factor VIII and coil embolization of the feeding arteries of the lesion on the CPA were performed, and the tumor was subtotally resected without hemorrhagic complications. The histopathological diagnosis was hemangioblastoma. We report this case to emphasize the importance not to overlook previously undiagnosed coagulopathy before surgical excision of hemangioblastoma. And, with appropriate perioperative management for coagulopathy, surgical treatment involving a high risk of perioperative bleeding can be safely undertaken.

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Surgery and Hemostasis

Lawson¹,

Kitchens²

2019

Consultative Hemostasis and Thrombosis

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Surgical Procedures in Unsuspected Hemophilia

Cited by 6 publications

References 4 publications

Health‐related quality of life in a cohort of adult patients with mild hemophilia A

Health‐related quality of life in a cohort of adult patients with mild hemophilia A

A Rare Case of Giant Solid Hemangioblastoma Accompanied with Hemophilia Type A

Surgery and Hemostasis

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