Systematic review of the presentation of coagulation factor VIII inhibitors in rheumatic diseases: A potential cause of life-threatening hemorrhage

O’Connor, Carolyn R.

doi:10.1016/j.semarthrit.2014.11.008

Cited by 19 publications

(21 citation statements)

References 95 publications

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“…It is also imperative to introduce an appropriate immunosuppressive treatment to eliminate the inhibitor in order to achieve complete remission, which is defined by normal factor VIII activity with an undetectable inhibitor [ 28 – 30 ]. The sustained response is considered to be maintained if the inhibitor is less than 0.6 BU/mL and the activity of factor VIII is greater than 50%, whereas partial remission is established by an inhibitor titer of less than 5 BU/mL and a factor VIII activity of 30% [ 31 ].…”

Section: Discussionmentioning

confidence: 99%

“…Especially for patients in geographic locations where other hemostatic alternatives are not readily available, human FVIII replacement could be a sufficient first-line therapy and has proven effective in some patients [ 34 ]. Other treatment regimens, including corticosteroid therapy, immunosuppressants, immunoglobulin, and coagulation factors having a factor VIII inhibitor short-circuiting activity, have been reported [ 10 , 31 , 35 – 37 ]. The results were often favorable, but a few deaths were reported, which were mainly due to the comorbidities associated with AH rather than the hemorrhagic syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature

et al. 2018

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Patient: Male, 66Final Diagnosis: Acquired hemophilia ASymptoms: Polyarticular flareMedication: —Clinical Procedure: —Specialty: RheumatologyObjective:Challenging differential diagnosisBackground:Acquired hemophilia A (AH) is a rare hemorrhagic diathesis, characterized by the presence of autoantibodies directed against the pro-coagulant activity of factor VIII. It is associated with rheumatoid arthritis (RA) in 4% to 8% of cases and its prognosis remains severe.Case Report:A 66-year-old patient has been followed up for 20 years for deforming and severe RA, which was in low-disease activity. However, the patient presented a polyarticular flare involving the metacarpophalangeal and the proximal interphalangeal joints, the left elbow, and the right knee, which was warm and swollen. Articular puncture of this knee yielded a hematic fluid that did not coagulate. Its cytological analysis showed significant presence of red blood cells, which were also abundantly present in the other cell lines. Activated partial thromboplastin time was lengthened and not corrected by the addition of control plasma. Prothrombin time (Quick’s test), fibrinogen level, and vitamin K-dependent factors were without abnormalities. In contrast, factor VIII was collapsed at 7% and the anti-factor VIII antibody was positive. The diagnosis of AH with anti-factor VIII inhibitor was thus retained. With regard to RA, the Disease Activity Score was 6.32 and exhibited a very active RA. Rituximab with methotrexate was begun and the evolution was favorable. After 6 months, the reappearance of the anti-factor VIII inhibitor was found, thus justifying a second cycle of rituximab.Conclusions:AH is not exceptional in RA. Rituximab remains a relevant alternative for managing simultaneous AH with inhibitor and RA.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature

et al. 2018

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“…Nearly 10% of patients with AHA have an underlying malignancy, either solid or haematologic [7,9] . AHA is also associated autoimmune disease; 4-8% of cases are associated with rheumatoid arthritis [10] . Patients with AHA are predisposed to developing antibodies and high-titre inhibitors, meaning that intense treatment is often required [7].…”

Section: Discussionmentioning

confidence: 99%

Acquired haemophilia A in an elderly patient related to relapsed cervix carcinoma

Atilla

Unat

Bülbül

et al. 2018

The Journal of Haemophilia Practice

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Acquired haemophilia A (AHA) is a rare, autoimmune disease, presenting as sudden haemorrhages without any personal or family history. Anti-factor VIII (FVIII) is the most commonly recognised autoantibody resulting in decreased factor activity. The aetiology and pathophysiology of these antibodies remains unclear. Approximately 50% of reported cases are idiopathic; the rest are associated with other conditions, mainly underlying malignancies, autoimmune diseases (eg rheumatoid arthritis (RA), systemic lupus erythematosus), drug administration and postpartum period. A 74-year-old woman presented to our institution with haematochezia and haematuria. She had a medical history of cervix carcinoma; total abdominal hysterectomy and bilateral salpingo-oophorectomy with postoperative chemoradiotherapy was performed in 2011. She had also been followed up for 20 years for deforming and severe RA, which was in low-disease activity with methotrexate and corticosteroid. Laboratory investigations for abnormal bleeding revealed prolongation of activated partial thromboplastin time (APTT). APTT prolongation was not corrected by 50:50 plasma mixing studies, and a confirmatory factor assay demonstrated FVIII deficiency (1.4 IU/dL; normal range 50-150 IU/dL). Positive FVIII antibodies on Bethesda testing confirmed a diagnosis of AHA. A rectosigmoid mass and fistula between rectum and bladder were discovered by computed tomography (CT). Bleeding was controlled with recombinant activated factor VII (rFVIIa) after two weeks. Eradication of the inhibitor was achieved with high-dose pulse methylprednisolone for two days and then 2mg/kg daily over four weeks.

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“…5 Approximately 50% of cases are idiopathic, though an association with malignancy and autoimmunity has been suggested. [5][6][7][8][9][10] Typical symptoms include subcutaneous, mucosal, and muscle bleeds; hemarthrosis is uncommon. 1,5,11 Little is known about pediatric AHA due to its exceedingly low incidence in children.…”

Section: Pediatric Acquired Factor VIII Deficiency Presenting As Hema...mentioning

confidence: 99%

Pediatric acquired factor VIII deficiency presenting as hemarthrosis

Daigh

Chien

2021

Pediatric Blood & Cancer

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Bleeding events are indicated with an asterisk. Intravenous immune globulin (IVIG) doses are indicated with an arrowhead. (a) Prednisone started at 1 mg/kg/dose twice daily; (b) prednisone taper initiated; (c) prednisone discontinued. (B) FVIII inhibitor levels and FVIII activity versus time since diagnosis in case #2. Bleeding events are indicated with an asterisk. (a) Prednisone started at 1 mg/kg/dose twice daily; (b) prednisone taper initiated with 5 mg/dose decrease each week; (c) prednisone discontinued vaccination. As demonstrated with these two cases, immunosuppression with steroids ± IVIG may be curative in pediatric AHA.

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Systematic review of the presentation of coagulation factor VIII inhibitors in rheumatic diseases: A potential cause of life-threatening hemorrhage

Cited by 19 publications

References 95 publications

Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature

Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature

Acquired haemophilia A in an elderly patient related to relapsed cervix carcinoma

Pediatric acquired factor VIII deficiency presenting as hemarthrosis

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