Acquired haemophilia A as a blood transfusion emergency.
BACKGROUND
Acquired haemophilia is a rare autoimmune disorder caused by autoantibodies directed in the majority of the cases against clotting factor VIII. This disorder is characterised by the sudden onset of bleeding that not rarely may be life-threatening and need transfusion support. Most reports on this condition describe the need for blood transfusions during the acute, haemorrhagic phase, but the number of transfused red cell units is often unknown.


METHODS
In the last 5 years, 14 patients with acquired haemophilia A were identified in the transfusion and haemophilia centres of Verona and Castelfranco Veneto. The transfusion support for these 14 patients was analyzed in this retrospective survey.


RESULTS
The 14 patients required a total of 183 red cell units. The average transfusion requirement was 13 red cells units/patient, with a range from 0 to 38 units.


CONCLUSIONS
Eleven of the 14 patients studied needed strong transfusion support to enable any further management of the haemorrhages, as well as for eradication treatment of the autoantibodies to factor VIII. A relevant part of the management of haemorrhagic symptoms as well as the first choice for any further treatment (bleeding or the cure of the underlying disease) is transfusion of red blood cells.
Tagariello
Giuseppe
G
Servizio Immunoematologia e Trasfusione, Centro Regionale per le Malattie del Sangue e Centro emofilia, ASL 8, Ospedale di Castelfranco Veneto, Castelfranco Veneto, Italy. giuseppe.tagariello@ulssasolo.ven.it
Sartori
Roberto
R
Radossi
Paolo
P
Risato
Renzo
R
Roveroni
Giovanni
G
Tassinari
Cristina
C
Giuffrida
Annachiara
A
Gandini
Giorgio
G
Franchini
Massimo
M
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood* / Q000150:complications / Q000188:drug therapy; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000188:drug therapy / Q000209:etiology; D003422:Critical Care; D017707:Erythrocyte Transfusion; D005169:Factor VIII / Q000276:immunology*; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D012189:Retrospective Studies
D001323:Autoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa