Acquired FVIII and FIX Inhibitors after Pregnancy: A Case Report

Köse, Murat; Bakkaloğlu, Oğuz Kağan; Amikishiyev, S.; Akpınar, Timur Selçuk; Saracoglu, Basak; Akcan, Tuğçe; Oktem, Melike; Yenerel, Mustafa Nuri; Güler, Kerim; Tükek, Tufan

doi:10.1159/000445706

Cited by 4 publications

(4 citation statements)

References 14 publications

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“…The diagnostic parameter of AHA is the coagulation profile indicating a defect in the intrinsic pathway and, as mentioned before, the hallmark is an isolated deranged aPTT. A study by Kose et al [7] has reported a case of postpartum AHA with an initial aPTT value of >120 seconds, which is the highest to be reported yet. Our case presented with an aPTT value of 90 seconds, which raised the suspicion of acquired hemophilia, which we confirmed by mixing studies.…”

Section: Discussionmentioning

confidence: 87%

“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Azam

Batool

Malik

et al. 2020

Cureus

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Acquired hemophilia A (AHA) is a bleeding diathesis caused by the production of autoantibodies to factor VIII (FVIII). It manifests as an isolated deranged activated partial thromboplastin time (aPTT) indicating a defect in the intrinsic coagulation pathway. Herein, we report a case of a 26-year-old woman who presented with hemoperitoneum in the postpartum period following a lower segment Caesarean section (LSCS). AHA carries significant mortality if it remains undiagnosed, and early recognition and measures to eradicate the acquired inhibitors are the mainstays of its management.

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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

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

Azam

Batool

Malik

et al. 2020

Cureus

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“…The most frequent bleeding events were obstetrical (43.3%), cutaneous (41.3% of reported cases), intramuscular (36.7%), and haemarthrosis (8%). Unusual sites were also reported, such as macrohaematuria ( n = 9), 23,31 haemothorax ( n = 2), 32,33 haematemesis ( n = 1), 16 prolonged bleeding after a venous or arterial puncture (3) 28,34,35 or dental procedure ( n = 6), 6,23,36 and a haemorrhagic corpus luteum ( n = 1) 37 . In 44 cases (29.3%), more than one type of bleeding occurred simultaneously.…”

Section: Resultsmentioning

confidence: 99%

Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review

et al. 2021

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The development of auto-antibodies against coagulation factor VIII (FVIII) is a rare event, known as acquired haemophilia A (AHA). The bleeding pattern of AHA patients can vary from superficial bruising to severe life-threatening bleeding. Delay in diagnosis and initiation of an appropriate treatment may increase morbidity. Therefore, awareness of this rare bleeding disorder is a key issue. The global incidence is subject to various estimates; the annual incidence lays between 1.3 and 1.5/million/year. 1,2 The demographic distribution shows two age peaks: older patients

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“…AHA-related hemorrhages are associated with high morbidity and mortality [3][4][5], and risk of bleeding persists in the presence of inhibitors. An underlying cause is detected in approximately 50% of cases including autoimmune diseases, malignancy, infections, postpartum complication and others [6][7][8][9][10]. The FVIII: C and the inhibitor titer could not be measured in many hospitals due to the limited facility and resources, particularly in developing countries.…”

Section: Introductionmentioning

confidence: 99%

Inhibitor eradication and bleeding management of acquired hemophilia A: a single center experience in China

Liu

et al. 2019

Hematology

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Objectives: Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies against coagulation factor VIII that leads to spontaneous bleeding. This study reports the clinical characteristics and treatment outcomes of a relatively sizable cohort of patients with AHA. Methods: We retrospectively analyzed the characteristics and outcomes of 42 patients with AHA diagnosed in our center from January 2014 through December 2018. Results: The FVIII activity (FVIII: C) was significantly suppressed (median 1.5%; interquartile range [IQR]: 0.9-3.5) by FVIII inhibitor (median 8 BU/mL; IQR: 4.0-16.0). Bypassing agents, PCC or FVIIa, were used in 14 patients for bleeding control without any adverse reaction; and most patients (90.5%, 38/42) were placed on immunosuppressive regimen, corticosteroid alone or in combination with cyclophosphamide. Patients treated with corticosteroids alone had a lower median inhibitor titer (8 BU/mL) than those treated with combination corticosteroids of cyclophosphamide (16 BU/mL) (p < 0.001). 97.4% (37/38) patients achieved complete remission (CR) after immunosuppression therapy, and the median time to CR in patients treated with corticosteroids alone was shorter than those with combination corticosteroids of cyclophosphamide (median 40 days; IQR: 31-65 vs. 51 days; IQR: 38-83, p = 0.301). 10 (26.3%) patients relapsed thereafter and were placed on combined corticosteroid and cyclophosphamide treatment, which yielded second remission in 8 patients (80%). Two patients died, one from uncontrolled post-surgical retroperitoneal hemorrhage and one from sepsis complicating corticosteroid therapy. Conclusion: The corticosteroid achieves a satisfactory outcome, particularly with low inhibitors titers; and combination of cyclophosphamide will facilitate remission in sever patients with high titers of inhibitors.

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Acquired FVIII and FIX Inhibitors after Pregnancy: A Case Report

Cited by 4 publications

References 14 publications

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

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

Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review

Inhibitor eradication and bleeding management of acquired hemophilia A: a single center experience in China

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