Major surgery in haemophilia. Prolonged substitution therapy for surgical treatment of haemophilic cyst and pathological fracture of the femur

Bloom, A. L.; Enoch, B. A.; Richards, H. J.

doi:10.1002/bjs.1800550210

Cited by 5 publications

(3 citation statements)

References 14 publications

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“…of fresh average normal plasma. One pack of cryoprecipitate was prepared from the plasma of one blood donation and contained on average 85 units of Factor VIII (Bloom and Emmanuel, 1968). The amount of Factor VIII in each dose of cryoprecipitate was assessed from this average value and the number of packs administered, and the in-uiuo effect was checked by pre-and post-infusion Factor-VIII assays.…”

Section: Methodsmentioning

confidence: 99%

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Inhibitor of Factor VIII in Mild Haemophilia

1969

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Summary. Two patients with mild haemophilia developed inhibitors of Factor VIII during replacement therapy to cover major surgery. The inhibitors inactivated not only transfused antihaemophilic factor but also the patient's own Factor VIII and temporarily converted them to severe haemophiliacs. The activity of the inhibitors rapidly declined, possibly as a result of steroid therapy, and both patients eventually regained their previously mild haemophilic state.

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Section: Methodsmentioning

confidence: 99%

“…This patient has been fully described before by Bloom, Enoch and Richards (1968). The patient was a 21-year-old student who had been known to have a mild bleeding tendency since the age of 10 months, but had had no spontaneous haemorrhages or haemarthroses.…”

Section: Case Reports Case Imentioning

confidence: 99%

Inhibitor of Factor VIII in Mild Haemophilia

1969

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“…Successful haemostasis for surgery in people with haemophilia was reported from different parts of the world in the late 1960s (Fessey & Meynell, 1967; Wilson & Staveley, 1967; Bloom et al , 1968; Kemp & Matthews, 1968; Kwa, 1968; Gaum, 1969). At that time, it was a significant step forward from a situation where minor injuries could lead to life threatening complications to a position where major elective surgery could be performed with adequate haemostasis.…”

Section: Surgery In Haemophilia – Optimal Dose For Haemostasismentioning

confidence: 99%

Dose and response in haemophilia – optimization of factor replacement therapy

Srivastava

2004

Br J Haematol

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SummaryThe mainstay of the management of haemophilia is the replacement of clotting factors, using clotting factor concentrates (CFC) in a way that prevents bleeding and its complications. Beginning with small doses, as whole blood and plasma over 50 years ago, highly purified CFCs are now administered frequently in large doses to effectively treat this condition so that even people with severe haemophilia can lead near normal lives. However, with such regimens, compliance and expense have both become significant issues. The question therefore is whether the current models of clotting factor replacement are optimal. This article reviews the literature on the dose-response relationship in haemophilia, with particular reference to management of musculoskeletal bleeding and surgical haemostasis. Current practices are based on uncontrolled observational data. Less intensive protocols could achieve similar outcomes. Large multi-centre prospective studies are needed to provide comparative data on unresolved issues so that factor replacement therapy can be optimized, based on evidence.

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Hemophilic pseudotumor: Multicenter experience over 25‐year period

et al. 1994

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Pseudotumor is an infrequent manifestation of bleeding diathesis seen in hemophiliacs, the management of which is still controversial. To ascertain the effectiveness of the main therapies of choice, we have reviewed 1,831 patients affected by hemophilias A (1,108) and B (172), von Willebrand's disease (329), and other miscellaneous coagulopathies (222) diagnosed between 1965 and 1990 in a multicentric, retrospective study. Pseudotumor was proven in 21 patients. Replacement therapy was given in 15 cases as the first therapeutic approach, with complete success attained in only two, whereas surgery, which was carried out in 14 patients, completely resolved the process in eight (P = 0.017). Patients over 40 years of age demonstrated worse prognosis than younger patients (P = 0.02), but no other clinical parameters were shown to have influenced the patients' evolution. Surgical management was the most effective treatment for pseudotumor, although more conservative therapies cannot be overlooked in selected cases.

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Major surgery in haemophilia. Prolonged substitution therapy for surgical treatment of haemophilic cyst and pathological fracture of the femur

Cited by 5 publications

References 14 publications

Inhibitor of Factor VIII in Mild Haemophilia

Inhibitor of Factor VIII in Mild Haemophilia

Dose and response in haemophilia – optimization of factor replacement therapy

Hemophilic pseudotumor: Multicenter experience over 25‐year period

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