A Survey of 215 Non-Hemophilic Patients with Inhibitors to Factor VIII

Abstract

SummaryInformation was obtained by questionnaire about 215 nonhemophilic patients who developed inhibitors against factor VIII (antihemophilic factor). The majority of the patients were over 50 years of age, and approximately equal numbers of males and females were reported. Rheumatoid arthritis was present in 8% of the cases, 7% occurred during pregnancy or the post-partum period, and in several there was an association with allergy to penicillin, asthma, “auto-immune” diseases, or malignancy. In 46% of cases, no underlying disorders were identified. Major bleeding was observed in 87 % of patients, and in 22%, death was attributed either directly or indirectly to the presence of the inhibitor.In 11 of 31 patients receiving no therapy other than supportive transfusions of blood or factor VIII concentrate, the inhibitor disappeared after being present for an average duration of 14 months. Corticosteriods were thought to be effective in abolishing the inhibitor in 22 of 45 patients in whom these were the only drugs administered. Twenty-eight patients received azathioprine as well as corticosteriods; in 19, the inhibitor declined or disappeared during treatment. Finally, 80 patients were treated with cyclophosphamide; in 37 there was a favorable outcome. Inhibitors in children and post-partum patients were more likely to disappear spontaneously or with steroid therapy, whereas those in patients with rheumatoid arthritis or other “autoimmune” disorders required treatment with alkylating agents. However, before any specific therapy can be recommended for this disorder, prospective trials of potential therapeutic agents should be conducted in selected subgroups.

Rare Case of Acquired Haemophilia and Lupus Anticoagulant

“…Such a form of acquired haemophilia is called idiopathic. Quite a significant percentage of acquired haemophilia patients (20 %) represent individuals with a coexisting autoimmune disease, usually systemic lupus erythematosus, but also with rheumatoid arthritis, ulcerative colitis or a lymphoproliferative disease [9]. 10 % of cases of acquired haemophilia occur postpartum.…”

Symptomatic Acquired Haemophilia Due to Circulating Antibodies Against Both Factor VIII and IX in a Non-haemophiliac Patient

“…Acquired hemophilia is a rare condition caused by occurrence of inhibitor antibodies to factor VIII or IX [1]. The estimated annual incidence is around 1-1.5 per million populations and acquired haemophilia A is the most common subtype [1].…”

“…The estimated annual incidence is around 1-1.5 per million populations and acquired haemophilia A is the most common subtype [1]. Acquired haemophilia B is extremely rare and only few cases have been described [2].…”

“…However in about 50 % of patients no causes are found and are labelled as idiopathic acquired haemophilia [1,3,4]. The common clinical manifestations include spontaneous haemorrhages into the skin, muscles or soft tissues or excessive bleeding during surgery with an extremely high mortality due to major bleeding episodes.…”

Acquired haemophilia in a patient with discoid lupus

“…Mortality is rather high in newly diagnosed acquired haemophilia ranging from 8 to 22 % in different studies [2][3][4][5]. A major cause of significant mortality and morbidity is the frequent confusion with other life-threatening conditions such as disseminated intravascular coagulation (DIC), thus often leading to uncontrolled bleeding prior to correct diagnosis of the disease.…”