The First Case of Postpartum Acquired Hemophilia A in Korea

Abstract

Acquired hemophilia A (AHA) is a rare coagulopathy caused by autoantibodies to coagulation factor VIII (FVIII). Most patients with AHA have been previously healthy; however, a variety of morbidities have been associated with the condition including pregnancy. A 40-yr-old woman visited our institution with extensive hematoma on the right hip area. Her medical history revealed no personal or familial history of bleeding diathesis. Her coagulation tests showed markedly prolonged aPTT (117 sec), markedly decreased level of FVIII activity (0.4%) and high-titer FVIII inhibitor (77 BU). Collectively, she was diagnosed as having postpartum AHA and was treated with bypassing agents and corticosteroids. Her aPTT was normalized on the 174th postpartum day and FVIII inhibitor showed negative conversion on the 224th postpartum day. This is the first case of postpartum AHA with high-titer FVIII inhibitor in Korea. Timely diagnosis and management can reduce morbidity and mortality of this potentially life-threatening condition.

Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report

“…Pregnancy-associated AHA represents 7-11% of the entire disease burden, with patients commonly presenting within one to four months of delivery with variable presentations of bleeding [ 1 ]. A review of the last 10 years of reported postpartum AHA cases showed that the ages of patients ranged between 19 years [ 7 ] to 40 years [ 8 ]. This indicates that AHA related to pregnancy can occur in any woman of childbearing age.…”

“…Our patient reported the occurrence of a massive hemorrhagic disease on the very first day post-LSCS, while Spencer et al [ 9 ] have reported the occurrence of massive hematomas in a primigravida five weeks post-LSCS. In another study, Lee et al [ 8 ] have reported a vaginal bleed five days after the LSCS, and the development of large hematomas 65 days after the LSCS. Up to 9% of cases present with retroperitoneal bleeds and the bleeding pattern in AHA differs from congenital hemophilia, where mostly joint bleeds are seen [ 10 ].…”

Acquired Hemophilia A

“…자가면역성 질환(전신홍반루푸스, 류마티스관절염 등), 악성종양, 임신 (대부분 첫 번째 임신에서 발생하며, 출산 후 1-4개월에 발생함. 자가항체 가 태반을 통과하므로 태아에 출혈 위험이 있음), 5,6 감염, 7 약제 등 후천성 혈우병 A 발생에 관련된 원인이 있는 경우가 50% 정도 되며, 나머지 50% 에서는 원인불명(idiopathic)으로 밝혀진 관련된 질환 없이 발생하는 것으 로 알려져 있다. 1,8 비록 약 30% 정도에서는 출혈이 심하지 않아 출혈에 대 한 지혈치료가 필요 없으나, 최근의 대규모 보고에 따르면 94.6%에서는 출 혈성 임상양상이 동반되었고, 77%의 자연출혈과 70%의 심각한 출혈(혈 색소 < 8 g/dL 또는 혈색소 >2 g/dL 감소로 정의됨)이 관찰되었다.…”

Second case of postpartum acquired hemophilia A in a Korean female

“…Postpartum AHA is a special category with distinct clinical manifestation contributing to 7-21% of patients with AHA [ 2 , 3 , 4 ]. In Korea, the first case of postpartum AHA in a 40 years old female has been reported by Lee et al [ 5 ]. To the best of our knowledge, the present case report is the second one in Korea.…”

“…In the previous Korean case of postpartum AHA reported by Lee et al [ 5 ], the patient was treated with a bypassing agent plus corticosteroid. In the present case, we are using on-demand FEIBA infusion for control of acute bleeding and postponed the immunosuppression considering a spontaneous remission.…”