Determining the approximate factor VIII level of patients with severe haemophilia A on emicizumab using in vivo global haemostasis assays

Haemophilia ◽  
2021 ◽  
Author(s):  
Hande Kizilocak ◽  
Elizabeth Marquez‐Casas ◽  
Jemily Malvar ◽  
Roxana Carmona ◽  
Guy Young
Keyword(s):  
Factor Viii ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Factor Viii Level ◽  
Viii Level

scholarly journals Flap cover in a patient with severe haemophilia type A

2017 ◽  
Vol 50 (02) ◽  
pp. 213-216
Author(s):  
Narender Manickavachakan ◽  
Sunderraj Ellur ◽  
Vijay Thomas Mattyoo Joseph ◽  
Jonathan Victor ◽  
Cecil R. Ross
Keyword(s):  
Factor Viii ◽  
Indian Subcontinent ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Successful Management ◽  
Factor Viii Level ◽  
Spontaneous Bleeding ◽  
Viii Level ◽  
The Right ◽  
Flap Cover

ABSTRACTHaemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is <1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to maintain adequate levels in the intraoperative, immediate and late post-operative periods, together with adjuvant pro-coagulants, can ensure a safe outcome following surgery. We describe the successful management of one such patient suffering from Marjolin's ulcer of the right gluteal region, who needed wide local excision followed by flap cover. A protocol for management of such patients is also suggested. This is the first such case report from the Indian subcontinent, with only a few such published reports from the West.

Factor-VIII Inhibitor in Less Severely Affected Haemophilic Patients

1975 ◽  
Author(s):  
E. G. D. Tuddenham ◽  
A. L. Bloom ◽  
J. C. Giddings ◽  
C. A. Barrett
Keyword(s):  
Factor Viii ◽  
Factor Viii Inhibitor ◽  
Factor Viii Level ◽  
Replacement Treatment ◽  
Viii Level ◽  
Viii Inhibitor ◽  
In Vivo Recovery ◽  
Better Than

The occurrence of factor VIII inhibitor in five mild or moderately affected liaemophilic patients is described. In four patients the inhibitor inactivated endogenous factor VIII an dtemporarily converted them to severely affected haemophiliacs with factor VIII level of 0%. In the fifth patient, a brother of one of the others, the inhibitor although more potent did not inactivate the patient’s own factor VIII and did not completely inactivate normal factor VIII in vitro. This patient responded to treatment with factor-VIII concentrate but the in-vivo recovery was reduced. The patient’s plasma was tested against a panel of normal donors but it inactivated factor VIII in each to a similar extent and no evidence for normal factor-VIII groups was obtained. In the other patients the response to replacement treatment was also better than that usually seen in severely affected haemophilic patients with inhibitor. In the two related patients the inhibitors have so far persisted but in the unrelated patients the inhibitors eventually disappeared and did not always recur with subsequent therapy. The incidence of factor- VIII inhibitor in less severe haemophiliacs (factor VIII > 3% ) in this centre is 6% suggesting that the complication is more frequent in this type of patient than hitherto recognised.

Undetected Factor VIII Level in a Type III von Willebrand Disease (vWD) Patient Masquerading as Severe Hemophilia A.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4095-4095
Author(s):  
Ali M. Mullah-Ali ◽  
Anthony Chan ◽  
Mohan K. Pai ◽  
Kay Decker ◽  
Karen A. Moffat ◽  
...  
Keyword(s):  
Dna Analysis ◽  
Initial Diagnosis ◽  
Von Willebrand Disease ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Type Iii ◽  
Measurable Level ◽  
Viii Level ◽  
Red Cell Transfusion

Abstract Type III vWD is a rare disorder characterized by absent or <0.01u/ml of ristocetin cofactor (vW:RCoF), and a Factor (F) VIII:C of 0.03–0.10 u/ml. A total absence of FVIII:C has never been reported in type III vWD. We wish to report one such case where there was no measurable level of FVIII:C in a child leading to an initial diagnosis of severe haemophilia A. This patient presented at birth with post circumcision bleeding that was controlled with local measures. A packed RBC transfusion of 12 ml/kg body weight was given to correct acute blood loss. Coagulation studies performed before red cell transfusion revealed a FVIII:C of <0.01u/ml leading to diagnosis of severe haemophilia A. Patient required no further treatment to control bleeding. At 14 months of age the patient presented with an intracranial hemorrhage (ICH). rAHF (KogenateFS®) in doses of 50 to 400 u/kg resulted in poor recovery, half-life of 88 minutes and an in vivo survival of less than 4 hours. There was no FVIII:C inhibitor but vWF:RCoF, vWF:Ag and vWF multimers were totally absent. vWF concentrate (Humate-P®) in doses of 50–100/u/kg as vW:RCoF resulted in good clinical response, appropriate in vivo recovery and survival as shown in graphs below. This patient has made an excellent recovery and is fully functional 8 weeks after the ICH. Further enquiry into the family history revealed consanguinity with marriage between first cousins. A female cousin is also affected with easy bruising. Both parents have normal FVIII:C and borderline low vWF:RCoF. DNA analysis of the parents, propositus and unaffected siblings is in progress. Our case illustrates the importance of excluding Type III vWD at initial diagnosis even when the FVIII:C is <0.01u/ml instead of assuming it to be a severe haemophilia A. Figure Figure Figure Figure

scholarly journals Severe haemophilia A in a neonate presenting as haemopneumothorax after tracheo-oesophageal fistula-oesophageal atresia repair

2018 ◽  
pp. bcr-2018-225526
Author(s):  
Zita Hung ◽  
Mohammed Bahari ◽  
Mark J Belletrutti ◽  
Chloe Joynt
Keyword(s):  
Factor Viii ◽  
Oesophageal Atresia ◽  
Male Infant ◽  
Fresh Frozen Plasma ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Intron 22 Inversion ◽  
Viii Level ◽  
Fresh Frozen ◽  
Frozen Plasma

A male infant with oesophageal atresia and distal tracheo-oesophageal fistula (TEF type C) underwent right thoracotomy and transpleural repair of TEF on day 4 of life. He did not have a family history of coagulation disorders. A preoperative finding of prolonged partial thromboplastin time (PTT)>200 s was overlooked, and he went to surgery. There were no concerns with haemostasis prior to and even during the operation. The prolonged PTT was treated with one 10 mL/kg dose of fresh frozen plasma in the immediate postoperative period. On the fourth postoperative day, the infant developed a right haemopneumothorax, requiring fresh frozen plasma and packed cell transfusions. He was subsequently diagnosed with severe haemophilia A due to intron 22 inversion in the factor VIII gene, with factor VIII level <0.01 IU/mL.

Adenoidectomy in a girl with haemophilia

1991 ◽  
Vol 105 (11) ◽  
pp. 957-958 ◽  
Author(s):  
H. C. Harrison ◽  
A. Lammi
Keyword(s):  
Factor Viii ◽  
Haemophilia A ◽  
Bleeding Tendency ◽  
Inherited Disease ◽  
Factor Viii Level ◽  
Asymptomatic Carriers ◽  
Viii Level ◽  
Males And Females

AbstractHaemophilia A is a sex-linked inherited disease in which those affected are usually males, and females are usually asymptomatic carriers. This paper presents a haemophilia A carrier who has a low factor VIII level first, to remind readers that females can have low factor VIII levels with consequent increased tendency to bleed; second, to stress the importance of routine questioning regarding a bleeding tendency in the patient or the patient's family and third, to illustrate the importance of pre-operative diagnosis to allow adequate correction of the defect so that surgery such as adenoidectomy can be undertaken with safety in such a patient.

Haemostatic Failure of Prothrombin Complex Concentrates during Elective Dental Procedure

1979 ◽  
Vol 42 (05) ◽  
pp. 1604-1614 ◽  
Author(s):  
Philip M Blatt ◽  
Arthur H Pearsall ◽  
Edgar G Givhan ◽  
Charles E Hallum ◽  
Harold R Roberts ◽  
...  
Keyword(s):  
Factor Viii ◽  
Surgical Procedure ◽  
Haemophilia A ◽  
Dental Procedure ◽  
Prothrombin Complex Concentrates ◽  
Factor Viii Level ◽  
S Factor ◽  
Viii Level

SummaryOne haemophilia A patient with an inhibitor was exposed to a graded surgical procedure and treated with Prothrombin Complex Concentrates (PCC). Hemostasis was not obtained until the inhibitor was neutralized and the patient�s factor VIII level was normal. This patient is presented to emphasize that PCC are not nearly as effective as once hoped in producing hemostasis in inhibitor patients. Consequently, major decisions should not be made presuming efficacy of these products in inhibitor patients.

Epsilon-Aminocaproic Acid Inhibits the Activity of Factor VIII Inhibitors in Patients with Severe Haemophilia A in vivo and in vitro

2000 ◽  
Vol 103 (2) ◽  
pp. 67-72 ◽  
Author(s):  
Kanjaksha Ghosh ◽  
Shrimati Shetty ◽  
Anil Pathare ◽  
Dipika Mohanty
Keyword(s):  
Factor Viii ◽  
Aminocaproic Acid ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Epsilon Aminocaproic Acid

DDAVP Stimulates Prostacyclin Production

1982 ◽  
Vol 47 (02) ◽  
pp. 122-123 ◽  
Author(s):  
J J F Belch ◽  
M Small ◽  
F McKenzie ◽  
P A Hill ◽  
G D O Lowe ◽  
...  
Keyword(s):  
Factor Viii ◽  
Normal Subjects ◽  
Facial Flushing ◽  
Factor Viii Level ◽  
Anti Platelet Agent ◽  
Viii Level ◽  
Significant Release ◽  
Prostacyclin Production

SummaryDes-amino-D-arginine vasopressin (DDAVP) stimulates the release of factor VIII and plasminogen activator from the vascular endothelium. An infusion of exogenous factor VIII given to haemophiliacs causes an increase in platelet activation. This activation does not occur after stimulating a rise in the patient's own factor VIII level caused by DDAVP infusion. We hypothesised therefore that DDAVP could also cause the endothelial release of prostacyclin (PGI2), a potent anti-platelet agent which would counteract the aggregating effect of factor VIII. To examine this possibility we studied the effect of DDAVP on prostacyclin release, as measured by its stable metabolite 6-oxo-PGFla, in vitro and in vivo. Rabbit aortic rings were incubated with different concentrations of DDAVP using saline as control. The supernatant was assayed for 6-oxo-PGFlct by radioimmunoassay. All concentrations of DDAVP gave a significant release of 6-oxo-PGF1α. Vasopressin was much less potent. When DDAVP was infused into haemophilic patients there was a significant increase in circulating 6-oxo-PGF1α levels immediately after the infusion. The facial flushing observed as a side-effect of DDAVP could therefore be prostacyclin-mediated. We confirmed this by abolishing the DDAVP induced flushing seen in normal subjects by prior treatment with aspirin which inhibits PGI2 formation.

Management of Haemophilia in Sweden

1976 ◽  
Vol 35 (03) ◽  
pp. 510-521 ◽  
Author(s):  
Inga Marie Nilsson
Keyword(s):  
Factor Viii ◽  
Total Population ◽  
Age Groups ◽  
Factor Ix ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
Human Fraction ◽  
Mild Haemophilia

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.

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