Acquired Hemophilia: A Report of 4 Cases

Case Series

Ann Hematol Oncol. 2016; 3(1): 1074.

Acquired Hemophilia: A Report of 4 Cases

Karimi M1*, Abdolkarimi B1,2, Majid Naderi 3 and Parand S1

¹Hematology Research Center, Shiraz University of Medical Sciences, Iran

²Department of Pediatrics, Iran

³Children and Adolescents Health Research Center, Zahedan University of Medical Sciences, Iran

*Corresponding author: Mehran Karimi, Department of Pediatric Hematology-Oncology, Hematology Research Center, Shiraz University of Medical Sciences, Nemazee Hospital, Shiraz, Iran

Received: January 20, 2016; Accepted: March 08, 2016; Published: March 10, 2016


Acquired hemophilia A is very rare and often causes life-threatening hemorrhage. We report 4 cases of this disorder in different settings. Case1 received activated protrombin concentrate complex (aPCC) and the result was excellent. Case 2 received IVIG and high dose factor ѴІІІ, prednisolon and azathioprin and long term follow-up was without bleeding. Case 3 was a postpartum acquired hemophilia A and received recombinant FVIIa, prednisolon, cyclophosphamide and she had complete response. Case 4 was a 74 year- old man diagnosed with renal cell carcinoma induced acquired coagulopathy. He died due to initial mismanagement with high dose plasma concentrated factor VIII. Acquired hemophilia A should be treated by combination of immunosuppressive agents, aPCC or rFVIIa. It should be taken into consideration that the degree of inhibitor levels has no direct relationship with severity of bleeding symptoms and high index of suspicion is necessary for early diagnosis.

Keywords: Acquired hemophilia A; Factor VIII inhibitor; Hemophilia; Immunosuppressive therapy


Acquired hemophilia A (AHA) is very rare disease, especially in children. It is caused by production of antibody against coagulation factor VIII. It is associated with mortality and morbidity, so early diagnosis is very crucial.

In the 6th-7th decade of life, AHA is diagnosed in both sexes equally [1]. The final diagnosis was based on the time-dependent inhibitor of factor VIII in plasma and low plasma level of factor VIII

The isolated prolongation of activated partial thromboplastin time (APTT) after the addition of normal plasma is the primary screening test. This situation was mistaken with other acquired bleeding disorders such as disseminated intra vascular coagulation. More than 50% of cases are idiopathic, but the underlying conditions including auto immune disease, malignancy, infection, pregnancy, medications (especially antibiotics, psychiatric drugs) and immune modulating agents can cause AHA [2]. The mortality rate is estimated from 7.9% to 22%. Therefore, accurate and early diagnosis and treatment can prevent mortality and morbidity. We reported four cases with AHA who were presented with different clinical settings.

Case Presentation

Case 1

The patient was a 13 year-old boy who presented with right elbow (Figure 1) and right knee joint edema secondary to mild trauma in school. Sonography of right elbow and knee showed hemorrhage. Laboratory evaluations were done and showed mild anemia (HB=10.7 gr/Dl) and prolonged partial thrombin time (PTT=80 second).