Acquired Hemophilia in Female - A Case Report

Israt Zerin,Khan Abul Kalam Azad,Madhabi Karmaker,Md Mujibur Rahman,Salma Afrose

doi:10.3329/jom.v18i2.33694

Abstract

Acquired hemophilia is a rare but acquired bleeding diathesis caused by autoimmune depletion of Factor VIII or IX. Diagnosis of Acquired Hemophilia requires clinical acumen. The condition is often under diagnosed as because it is rare and patients usually dont have any unusual personal or family history of bleeding episodes. Acquired hemophilia has an equal sex distribution. So, clinicians should suspect acquired hemophilia in any patients with unexplained persistent and profound bleeding from uncommon soft tissues and mucosa and having prolonged activated partial thromboplastin time (APTT) without other causes. Here we will report a 50-year-old lady presenting with recurrent spontaneous bruises and joint pain and swelling for 2 years. On examination we found multiple bruises and ecchymosis of different sizes with varying colour changes from bluish to greenish over different parts of her body including face, right shoulder, right elbow joint and 2nd and 3rd PIP joints. Lab findings showed decreased Hemoglobin with prolonged APTT which didnt correct in mixing study. After exclusion of other possible pathological conditions and on the basis of lab criteria we diagnosed the case as Acquired Hemophilia. She was successfully treated with blood transfusion, recombinant activated factor VIII, Steroid and supportive treatments and was discharged with improving symptoms, stable hematocrit and resolving bruises.J MEDICINE July 2017; 18 (2) : 119-122

Highlights

Acquired Hemophilia (AH) is a rare condition in which auto antibodies, usually of the IgG class are produced against factor VIII or IX
Most antibodies bind to the 44DA2 domain and/or the 72 KDC2 domain of factor VIII which is associated with insufficient generation of thrombin through intrinsic pathway of coagulation cascade and increased bleeding diathesis
The incidence of Acquired Hemophilia increases with age with a peak incidence in 7th and 8th decades of life though there is a small peak in the 3rd decade likely corresponding to post partum inhibitors

Summary

Introduction

Acquired Hemophilia (AH) is a rare condition in which auto antibodies, usually of the IgG class are produced against factor VIII or IX. Last episode occurred 1 day before admission when her whole face became enormously swollen with bluish black discoloration after a slap over her face by her husband At that time she presented with myalgia and painful swelling of right shoulder joint, right elbow joint, and 2nd and 3rd PIP joints of right hand with overlying bruises without any precipitating illness or trauma. On further enquiry, she complained of gum bleeding while brushing her teeth very rarely over last 20 years, increased menstrual flow and duration of 8-9 days with regular cycle with normal frequency over 2 years with no previous menstrual abnormality.

Discussion

Von willebrand disease and

Conclusion