Acquired hemophilia A: three different presentations of the same disease.
Acquired hemophilia A is a rare disorder characterized by the presence of an autoantibody (mainly immunoglobulin G) to the clotting factor VIII with a clinical resemblance to hemophilia A. This autoantibody may arise because of dysregulation of the immune system. It is associated with various autoimmune or dermatologic diseases, pregnancy, or drug ingestion, but in almost 50% patients, the cause is unknown. In the present study, we have reported three different clinical presentations of acquired hemophilia. In two cases, the underlying disorder was the probable respiratory chronic disease (asthma), and in the other, it was idiopathic. We reviewed the response to a given treatment. The severity of the clinical presentation was different in all the cases, and was taken into account when we decided on the best course of treatment. The present report presents two patients successfully treated with a tapering course of steroids, and one with the anti-CD20 monoclonal antibody not given as first line treatment.
del Campo
Raquel
R
Servicio de Hematologia y Hemoterapia, Hospital Son Llatzer, Carretera de Manacor km 4, Palma de Mallorca, Balearic Islands, Spain. rcampo@hsll.es
Bargay
Juan
J
Cladera
Antonia
A
Balaguer
Humbelina
H
Requena
Maria José
MJ
Mascaró
Martin
M
D000368:Aged; D001247:Asthenia / Q000150:complications* / Q000188:drug therapy; D001249:Asthma / Q000150:complications* / Q000188:drug therapy; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006406:Hematoma / Q000150:complications* / Q000601:surgery; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D012771:Shock, Hemorrhagic / Q000188:drug therapy; D013256:Steroids / Q000627:therapeutic use
D011994:Recombinant Proteins; D013256:Steroids; C103587:recombinant FVIIa; D015942:Factor VIIa