scholarly journals Acquired haemophilia: a case report and a clinical review

2013 ◽  
pp. 47-49
Author(s):  
Lucia Todaro ◽  
Giuseppe Leone ◽  
Mauro Girotto ◽  
Pierdomenico Bertello
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Medical Emergency ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Laboratory Findings ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii ◽  
Prolonged Aptt

BACKGROUND Acquired haemophilia presents later in life and is due to acquired inhibitors to factor VIII. The disorder is very rare (about one case per million population per year) but causes significant morbidity and mortality. CASE REPORT We report the case of a 57 year-old obese man who was admitted to our Day Hospital for anemia. At admission we observed diffuse ecchymoses and a large subcutaneous hematoma in the inferior right hemithorax. Laboratory findings showed severe anemia, a prolonged aPTT with a normal PT and platelet count. The titer of anti-factor VIII inhibitor in Bethesda units resulted very high and confirmed the diagnosis of acquired haemophilia. We used recombinant activated factor VII (rFVIIa) to control active bleeding and prednisone, cyclophosphamide and, lastly, the anti- CD 20 monoclonal antibody (rituximab) to eliminate the factor VIII inhibitors. Unfortunately, on fourth week, the patient suffered an extensive hematoma of the abdominal wall and died. CONCLUSIONS We underline the importance of not overlooking a prolonged aPTT because the bleedings associated with acquired haemophilia can be life-threatening and constitute a medical emergency.

Factor VIII Inhibitor Bypass Activity and Recombinant Activated Factor VII in Cardiac Surgery

2014 ◽  
Vol 28 (5) ◽  
pp. 1221-1226 ◽  
Author(s):  
Vidya K. Rao ◽  
Robert L. Lobato ◽  
Blake Bartlett ◽  
Mark Klanjac ◽  
Christina T. Mora-Mangano ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Factor Viii ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Viii Inhibitor

Elective Surgery on Factor VIII Inhibitor Patients Using Continuous Infusion of Recombinant Activated Factor VII

2001 ◽  
Vol 86 (10) ◽  
pp. 949-953 ◽  
Author(s):  
Christopher Ludlam ◽  
Peter Collins ◽  
Charles Hay ◽  
Jonathan Wilde ◽  
Alessandro Grigeri ◽  
...  
Keyword(s):  
Factor Viii ◽  
Continuous Infusion ◽  
Infusion Rate ◽  
Elective Surgery ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Fixed Rate ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Plasma Factor

SummaryWe examined recombinant activated factor VII (rVIIa) administered by continuous infusion to eight patients with inhibitors to factor VIII, undergoing elective surgery. rVIIa was infused at a fixed rate of 16.5 μg/kg/h for a median of 13.5 days (range 1-26). There was effective haemostasis at this infusion rate in only one of two minor procedures and two of six major operations. Three patients experienced excessive bleeding despite plasma factor VII activity around 10 IU/ml. Serious bleeding occurred in two other patients caused by procedural errors unrelated to rVIIa and required re-operation. The median rVIIa clearance on day 1 was 57 ml/h/kg (range 18-100) and on day 3 was 100 ml/h/kg (range 61-200). Clearance on the final infusion day was not significantly different from day 3. The infusion did not induce pathological activation of the coagulation mechanism. The only thrombotic adverse events were two episodes of superficial thrombophlebitis of the infused vein in one subject. In conclusion, the 16.5 μg/kg/h infusion rate reliably achieves plasma factor VII activity levels of 10 IU/ml, but this level does not provide reliable haemostasis.

Acquired von Willebrand syndrome 2004: International Registry

2004 ◽  
Vol 24 (01) ◽  
pp. 50-55 ◽  
Author(s):  
U. Budde ◽  
J. H. Rand ◽  
A. B. Federici
Keyword(s):  
Factor Viii ◽  
Myeloproliferative Disorders ◽  
Von Willebrand Disease ◽  
Factor Vii ◽  
Laboratory Findings ◽  
Recombinant Activated Factor Vii ◽  
Acquired Von Willebrand Syndrome ◽  
Activated Factor Vii ◽  
International Registry ◽  
Von Willebrand

SummaryThe acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease. Unlike the congenital form, AVWS usually occurs in individuals with no personal or family history of bleeding. Large studies on AVWS are not available, diagnosis remains difficult and treatment empirical. Acquired von Willebrand syndrome is especially frequent in lympho- or myeloproliferative disorders. It is associated with solid tumours, immunological and cardiovascular disorders as well as other miscellaneous conditions. Diagnosis of AVWS is based on assays measuring ristocetin cofactor activity or collagen binding, which are usually abnormally low, while factor VIII coagulant activity is sometimes within the reference range. FVIII/VWF inhibiting activities are found in only a minority of cases. Bleeding episodes in patients with AVWS are mostly of the mucocutaneous type and can be managed with desmopressin, plasma-derived factor VIII/von Willebrand factor (FVIII/VWF) concentrates and intravenous immunoglobulin. Recombinant activated factor VII can be useful in cases unresponsive to standard therapy. In conclusion, the AVWS, although rare, is certainly underestimated in clinical practice: The actual clinical impact of AVWS should be evaluated by prospective studies. The authors are co-ordinating an updated version of the International Registry on AVWS that will allow data to be entered directly online.

scholarly journals Acquired hemophilia A: A rare cause of gross hematuria

2015 ◽  
Vol 9 (11-12) ◽  
pp. 905
Author(s):  
Gregory W Hosier ◽  
Ross J Mason ◽  
K Sue Robinson ◽  
Gregory G Bailly
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Rare Condition ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Viii Inhibitor

Acquired hemophilia A is a rare condition caused by spontaneous development of factor VIII inhibitor. This condition most commonly presents with multiple hemorrhagic symptoms and isolated hematuria is exceedingly rare. Early diagnosis is important, as this condition carries a high mortality rate (13‒22%). We present a case of an 82-year-old man with isolated hematuria caused by a factor VIII inhibitor who was successfully treated with recombinant activated factor VII concentrate, as well as prednisone and cyclophosphamide.

The use of recombinant activated factor VII in patients with acquired haemophilia

Blood Reviews ◽  
2015 ◽  
Vol 29 ◽  
pp. S19-S25 ◽  
Author(s):  
Andreas Tiede ◽  
Kagehiro Amano ◽  
Alice Ma ◽  
Per Arkhammar ◽  
Soraya Benchikh el Fegoun ◽  
...  
Keyword(s):  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii

Antithrombin Affects Hemostatic Response to Recombinant Activated Factor VII in Factor VIII Deficient Plasma

2008 ◽  
Vol 106 (3) ◽  
pp. 719-724 ◽  
Author(s):  
Fania Szlam ◽  
Taro Taketomi ◽  
Chelsea A. Sheppard ◽  
Christine L. Kempton ◽  
Jerrold H. Levy ◽  
...  
Keyword(s):  
Factor Viii ◽  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii

Prophylactic use of a recombinant activated factor VII in delivery haemorrhage by caesarean in a woman with major factor VII deficiency: a case report

2011 ◽  
Vol 69 (6) ◽  
pp. 713-719
Author(s):  
Jean-François Comes ◽  
Jean Devignes ◽  
Olivier Thiebaugeorges ◽  
Marie-Elisabeth Briquel ◽  
Thomas Lecompte
Keyword(s):  
Case Report ◽  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Factor Vii Deficiency ◽  
Activated Factor Vii ◽  
Prophylactic Use
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