Vol 4, No 1 (2013)
Case report
Published online: 2013-04-10
Uncontrolled postoperative bleeding in woman with pemphigus and undiagnosed acquired haemophilia A
Hematologia 2013;4(1):71-75.
Abstract
Acquired haemophilia A (AHA) is caused by sudden appearance of autoantibodies against factor VIII (FVIII). The disease presents with severe or life-threatening haemorrhage in patients with no
personal history of bleeding. The mortality in AHA patients is estimated at even 22%. A 55-year-old female was admitted to the local hospital due to exacerbation of pemphigus foliaceus. After admission she presented rapid deterioration of general condition. Laboratory tests revealed rapidly increasing anaemia and prolongation of activated partial thrombin time (APTT). Truncal CT-scan showed extensive haematomas localized intra-abdominally as well as within left iliac and obturatormuscles. The patient received fresh frozen plasma (FFP) followed by surgical intervention. Additionally, an ovarian cyst was removed. Due to unlocalized intraoperative bleeding from left
subphrenic area a formal splenectomy was performed. The uncontrolled bleeding from postoperative wound was observed after surgery. Daily FFP transfusions did not reduce blood loss and the
APTT was not corrected. Detailed hematological tests revealed decreased factor VIII activity to 20 IU/dl and the presence of antibodies against factor VIII in the titer of 1.8 BU/ml. The AHA
was diagnosed. To control the bleeding recombinant FVIIa was used successfully. Synchronously, the immunosupressive treatment was administered. Due to recurrent bleeding the treatment with
by-passing agents was continued for 39 days. During therapy urinary cutaneous fi stula was observed. In the control CT-scan a persistent intraabdominal and intramuscular haematomas were presented (localized similarly as before the treatment). Six weeks of immunosupressive therapy eradicated the FVIII inhibitor. After eradication of FVIII inhibitor a surgical bloodless removal of hematomas and open bladder reconstruction were performed. The additional transfusions of red blood cells and fresh frozen plasma were not necessary. In conclusion, in patients presenting spontaneous bleeding to muscles and/or retroperitoneal space we suggest the delaying of surgical intervention until the detailed coagulation tests have been performed. Ideally, patients diagnosed with AHA should not undergo surgical interventions.
personal history of bleeding. The mortality in AHA patients is estimated at even 22%. A 55-year-old female was admitted to the local hospital due to exacerbation of pemphigus foliaceus. After admission she presented rapid deterioration of general condition. Laboratory tests revealed rapidly increasing anaemia and prolongation of activated partial thrombin time (APTT). Truncal CT-scan showed extensive haematomas localized intra-abdominally as well as within left iliac and obturatormuscles. The patient received fresh frozen plasma (FFP) followed by surgical intervention. Additionally, an ovarian cyst was removed. Due to unlocalized intraoperative bleeding from left
subphrenic area a formal splenectomy was performed. The uncontrolled bleeding from postoperative wound was observed after surgery. Daily FFP transfusions did not reduce blood loss and the
APTT was not corrected. Detailed hematological tests revealed decreased factor VIII activity to 20 IU/dl and the presence of antibodies against factor VIII in the titer of 1.8 BU/ml. The AHA
was diagnosed. To control the bleeding recombinant FVIIa was used successfully. Synchronously, the immunosupressive treatment was administered. Due to recurrent bleeding the treatment with
by-passing agents was continued for 39 days. During therapy urinary cutaneous fi stula was observed. In the control CT-scan a persistent intraabdominal and intramuscular haematomas were presented (localized similarly as before the treatment). Six weeks of immunosupressive therapy eradicated the FVIII inhibitor. After eradication of FVIII inhibitor a surgical bloodless removal of hematomas and open bladder reconstruction were performed. The additional transfusions of red blood cells and fresh frozen plasma were not necessary. In conclusion, in patients presenting spontaneous bleeding to muscles and/or retroperitoneal space we suggest the delaying of surgical intervention until the detailed coagulation tests have been performed. Ideally, patients diagnosed with AHA should not undergo surgical interventions.
Keywords: aquired haemophilia Afactor VIII inhibitorrecombinant activated factor VII
