Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A

Knöbl, Paul

doi:10.1007/s40265-018-1027-y

Cited by 37 publications

(48 citation statements)

References 64 publications

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“…While most cases are reported in adults and mainly elderly people with the median age of 60 to 67 years, there have been cases reported ranging from two years to 89 years of age also. A small population of women can develop factor VIII inhibitors during pregnancy [3][4].…”

Section: Discussionmentioning

confidence: 99%

An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria

Mehta

Reddivari

2020

Cureus

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A 90-year-old male with a past medical history of hypertension, chronic kidney disease stage II, and hyperlipidemia presented with complaints of intermittent hematuria. He had no prior history of hematuria or mucosal bleeds and denied having any trauma. His activated partial thromboplastin time (aPTT) was found to be mildly prolonged at 48.4 seconds and his factor VIII level was found to be very low at less than 3%.

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Section: Discussionmentioning

confidence: 99%

An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria

Mehta

Reddivari

2020

Cureus

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“…It is most frequently seen in the elderly population, and the median age of diagnosis is 75 years [2]. Males and females are equally affected; however, more cases are reported in women of childbearing age (20-40 years) related to pregnancy [8][9]. Most of the cases are idiopathic; however, several risk factors are implicated in AHA, the most common being pregnancy, post-partum status, and autoimmune disorders.…”

Section: Discussionmentioning

confidence: 99%

“…In patients with low factor VIII inhibitor titers (<5 BU) replacement with human factor VIII concentrates± DDAVP administration is recommended [3][4][5][6][7][8][9][10][11][12][13]. If the patient's antibody titer is high (>5 BU), replacement with even high doses of human factor VIII concentrates, is ineffective, as the inhibitor will immediately block all the factor VIII administered, therefore these patients are managed with bypassing agents [9]. Bypassing agents consist of activated prothrombin complex concentrates (APCC) and recombinant factor VIIa (rFVIIa) with an efficacy of 86% and 95%, respectively [3].…”

Section: Discussionmentioning

confidence: 99%

Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder

Singh¹,

Lubana²,

Dabrowski³

2020

Cureus

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Acquired hemophilia A (AHA) is a rare autoimmune hematological disorder that has an incidence of about 1.5 cases per million people per year. It occurs in the elderly with the median age of 75 years, and most of the cases are idiopathic. It occurs due to the development of factor VIII inhibitor, which is an autoantibody against factor VIII leading to potentially lifethreatening bleeding episodes. The diagnosis of AHA is often delayed and challenging. We report a case of an 86-year-old male who initially presented with signs and symptoms of a stroke. He was found to have oral mucosal bleeding and swelling of the floor of the mouth. He later developed epistaxis, hematuria, and melena. He had an isolated elevation of activated partial thromboplastin time (APTT) with very high levels of factor VIII inhibitor (1152 Bethesda units) and very low levels of Factor VIII (<1%). He was managed with supportive transfusion, bypass agents, and immunosuppressive therapy. AHA is a rare autoimmune bleeding disorder and is more commonly seen in the elderly population. Bleeding in AHA is usually sudden and sometimes life-threatening. Hence early hemostasis with bypassing agents and treatment with immunosuppressive agents should be initiated. Due to the rarity of the disorder, it is crucial to report AHA cases to create awareness and increase the index of suspicion of the clinicians for early diagnosis and treatment to prevent morbidity and mortality.

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“…An early recognition and diagnosis are critical to the management of AHA. Besides prompt control of bleeding, eradication of FVIII inhibitors is essential to the cure of AHA [20]. FVIII replacement is not able to rescue bleeding in most patients with high titers of inhibitors and bypassing agents are the only effective option under such circumstances.…”

Section: Discussionmentioning

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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Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A

Cited by 37 publications

References 64 publications

An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria

An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria

Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder

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

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