Acquired Hemophilia

نویسنده

  • Şinasi Özsoylu
چکیده

To the Editor, I would like to highlight 3 of our patients, a 14-year-old boy and females of 4 and 41 years old [1,2] with acquired hemophilia B seen at İhsan Doğramacı Children’s Hospital (previously Hacettepe) between 1963 and 1973 among 343 patients with hemophilia [3] on account of the case of acquired hemophilia A in a 78-year-old man who was successfully treated with a combined immunosuppressive and immunoadsorption approach by Bilgin et al. as reported in a recent issue of this journal [4]. Two of our 3 patients with acquired hemophilia B, which is rarer than acquired hemophilia A, improved most likely due to corticosteroid administration in at least one case and without any intervention in the others. We have shown antibodies against factor VIII in more than 20% of patients with X-linked hemophilia A and extremely rarely in normal people in low titers [3]; however, very severe bleeding due to the presence of AHG antibodies was not frequent. On this occasion I would rather use the term ‘hereditary’ instead of ‘congenital’ hemophilia, which was used by Bilgin et al., since inheritance is not involved in most congenital disorders, such as congenital tuberculosis, syphilis, CMV, rubella infection, etc. May I also indicate that methyl prednisolone at 1 mg/kg/day may not be effective as an immunosuppressive, but 30 mg/kg/day seems to be more effective? Conflict of Interest Statement The authors of this paper have no conflicts of interest, including specific financial interests, relationships, and/or affiliations relevant to the subject matter or materials included.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Myelofibrosis and acquired hemophilia A: a case report

BACKGROUND Myelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described. CASE PRESENTATION A 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated p...

متن کامل

Acquired hemophilia A: an underdiagnosed, severe bleeding disorder.

Acquired hemophilia is a rare bleeding disorder caused by autoantibodies that inhibit coagulation factor VIII. In most cases, it manifests with severe, often life‑threatening bleeds. Acquired hemophilia may be idiopathic or secondary to another condition, most commonly other autoimmune disease or cancer. Treatment is directed to stop bleeding and eradicate inhibitory autoantibodies. Like in mos...

متن کامل

What´s New in Orthopedic Surgery for People with Hemophilia

Modern orthopedic surgery can improve the musculoskeletal problems of people with hemophilia. The most commonly affected joints are the knees, the ankles and the elbows. The most common orthopedic procedures that PWH undergo are the following: RS, arthroscopic synovectomy, arthroscopic joint debridement, ankle arthrodesis, total joint arthroplasty and removal of pseudotumors. Every surgical pro...

متن کامل

Mechanism of factor VIIa-dependent coagulation in hemophilia blood.

The ability of factor VIIa to initiate thrombin generation and clot formation in blood from healthy donors, blood from patients with hemophilia A, and in anti-factor IX antibody-induced ("acquired") hemophilia B blood was investigated. In normal blood, both factor VIIa-tissue factor (TF) complex and factor VIIa alone initiated thrombin generation. The efficiency of factor VIIa was about 0.0001 ...

متن کامل

HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Mechanism of factor VIIa–dependent coagulation in hemophilia blood

The ability of factor VIIa to initiate thrombin generation and clot formation in blood from healthy donors, blood from patients with hemophilia A, and in anti–factor IX antibody–induced (“acquired”) hemophilia B blood was investigated. In normal blood, both factor VIIa–tissue factor (TF) complex and factor VIIa alone initiated thrombin generation. The efficiency of factor VIIa was about 0.0001 ...

متن کامل

Postpartum Acquired Hemophilia Factor VIII Inhibitors and Response to Therapy

Postpartum-acquired hemophilia A is a rare and potentially severe complication of pregnancy caused by an autoantibody against factor VIII [1]. Acquired factor VIII deficiency is associated with autoimmune conditions, neoplastic diseases, drug hypersensitivity, and pregnancy. A retrospective study analyzed 51 published cases of postpartum factor VIII inhibitors, with regard to the outcome accord...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 31  شماره 

صفحات  -

تاریخ انتشار 2014