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001-es BibID:BIBFORM077222
Első szerző:Árokszállási Anita (orvos)
Cím:A decade-long clinical experience on the prophylactic use of activated prothrombin complex concentrate in acquired haemophilia A : a case series from a tertiary care centre / Árokszállási Anita, Rázsó Katalin, Ilonczai Péter, Oláh Zsolt, Bereczky Zsuzsanna, Boda Zoltán, Schlammadinger Ágota
Dátum:2018
ISSN:0957-5235
Megjegyzések:In acquired haemophilia A (AHA), risk for recurrent bleeding exists until the inhibitor is detectable. Thus, patients with persisting inhibitor may benefit from prophylaxis with activated prothrombin complex concentrate (aPCC). Potential thromboembolic complications and cost are also factors to consider. Today, no high level evidence or clear recommendations are available on aPCC prophylaxis in AHA. Recently, a small prospective study demonstrated a favourable outcome with short-term, daily administered aPCC infusion. Here we report a retrospective case series of 19 patients with AHA to demonstrate our practice on aPCC prophylaxis. In our practice, clinical bleeding tendency guided our decision on the initiation of aPCC prophylaxis. In patients with serious bleeding tendency, aPCC infusion was prolonged beyond bleeding resolution in a twice-weekly or thrice-weekly regimen. Serious bleeding phenotype included a single episode of life-threatening bleeding or recurrent, severe haemorrhages. Patients who did not present such events were treated on-demand. The preventive dose of aPCC was equal with the lowest effective therapeutic dose. Prophylaxis was continued until the inhibitor disappeared. Eleven patients received aPCC prophylaxis. In nine cases, prophylaxis lasted beyond two months. No severe bleeding developed spontaneously and no thromboembolic complication occurred in the median 16 weeks (interquartile range 9-34) duration of prophylaxis. Eight patients of the nonprophylaxis group did not present any severe haemorrhage. According to our experience, we consider prophylaxis with aPCC effective and well tolerated for patients with AHA and serious bleeding tendency, until the acquired inhibitor persists.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
acquired haemophilia A
activated prothrombin complex concentrate
bleeding tendency
prophylaxis
Megjelenés:Blood Coagulation & Fibrinolysis. - 29 : 3 (2018), p. 282-287. -
További szerzők:Molnárné Rázsó Katalin (1966-) (belgyógyász, haematológus, klinikai onkológus) Ilonczai Péter (1977-) (orvos, belgyógyász, haematológus szakorvos) Oláh Zsolt (1974-) (belgyógyász) Bereczky Zsuzsanna (1974-) (orvosi laboratóriumi diagnosztika szakorvos) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Schlammadinger Ágota (1971-) (belgyógyász, haematológus)
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DOI
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001-es BibID:BIBFORM037155
Első szerző:Árokszállási Anita (orvos)
Cím:Acquired haemophilia : an often overlooked cause of bleeding - experience from a Hungarian tertiary care centre / Árokszállási Anita, Ilonczai Péter, Rázsó Katalin, Oláh Zsolt, Bereczky Zsuzsanna, Boda Zoltán, Schlammadinger Ágota
Dátum:2012
ISSN:0957-5235
Megjegyzések:Acquired haemophilia is a potentially life-threatening bleeding disorder. Its early diagnosis and treatment is of major importance. We evaluated the elapsed time between the first presentation of the bleeding symptoms and the correct diagnosis in the cases of the acquired haemophilia patients referred to our centre between 1999 and 2011. The causes and consequences of the often delayed diagnosis were also examined. The clinical and laboratory data of 13 patients with acquired haemophilia were analysed. Eleven patients had inhibitors to factor VIII (FVIII), in one case the autoantibody developed to factor XIII (FXIII) and in one to factor V (FV). The median period between the onset of the bleeding symptoms and the correct diagnosis was 1.5 months (3.0 days-9.0 months). In four cases 4.0-9.0 months were needed to establish the diagnosis. The main reason of this delay was that either the prothrombin time was used exclusively to evaluate haemostasis in primary care and also in some secondary care centres, or the prolonged activated partial thromboplastin time went unnoticed despite the obvious bleeding symptoms. Our observation underlines the importance of early referral of patients with unexplained bleeding symptoms to centres with appropriate laboratory facilities and experience in the diagnosis of bleeding disorders.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Blood Coagulation & Fibrinolysis. - 23 : 7 (2012), p. 584-589. -
További szerzők:Ilonczai Péter (1977-) (orvos, belgyógyász, haematológus szakorvos) Molnárné Rázsó Katalin (1966-) (belgyógyász, haematológus, klinikai onkológus) Oláh Zsolt (1974-) (belgyógyász) Bereczky Zsuzsanna (1974-) (orvosi laboratóriumi diagnosztika szakorvos) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Schlammadinger Ágota (1971-) (belgyógyász, haematológus)
Internet cím:DOI
Intézményi repozitóriumban (DEA) tárolt változat
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