Unexplained hemorrhagic syndrome? Consider acquired hemophilia A or B

Acquired hemophilia A (AHA) is an ultra-rare autoimmune disorder caused by autoantibodies against factor VIII. It often presents with life-threatening bleeding to non-hemophilia experts, who have limited awareness of this condition. This review evaluated hemostatic management and identified barriers to optimal management of AHA by non-hemophilia experts in the United States through a literature review. AHA case reports published by non-hemophilia experts from January 2016 through November 2021 in non-hematology journals were critically reviewed for a chronology of clinical course and management, consultation with a hemophilia expert, referencing of available AHA recommendations, discussion of all hemostatic options, and bleed control outcomes; 24 case reports representing 24 patients were identified. Twelve patients had an apparent delay in diagnosis, 17 cases did not seek expert consultation, and 15 did not reference the 2009 International AHA Recommendations, including six in whom hemostatic treatment was not consistent with the recommendations. Of the 17 articles published after the 2017 AHA Guidance, eight did not reference them. Of the five articles published after the 2020 International Recommendations for AHA, three did not reference them. Overall, 14 articles did not discuss all available hemostatic treatment options. Four patients died. Our findings reveal variability in hemostatic management of AHA by non-hemophilia experts in the United States. Lack of AHA awareness remains a primary barrier for optimal management of AHA among non-hemophilia experts. Increasing education about existing AHA guidelines, including available therapies and access to expert care at hemophilia treatment centers, may help improve the outcomes of patients with AHA.

/pdf/review-of-potential-barriers-to-effective-hemostatic-m65e5dzd.pdf

Review of Potential Barriers to Effective Hemostatic Management of Acquired Hemophilia A by Non-Hemophilia Experts in the United States

Acquired hemophilia A (AHA) is a rare disease that results from factor VIII inhibitors causing abnormal coagulation, and certain cases may develop after highly invasive surgery. The present case study reports on a 68-year-old male patient who developed AHA after undergoing a subtotal stomach-preserving pancreatoduodenectomy for distal cholangiocarcinoma. The patient experienced complications after surgery, requiring reoperation on postoperative day (PD) 5 due to rupture of the Braun's enterostomy. On PD 6, angiography was performed after bleeding was detected in the jejunal limb, but hemostasis occurred spontaneously during the examination. Bleeding was observed again on PD 8 and direct surgical ligation was performed. On PD 14, bleeding recurred in the jejunal limb and angiography was performed to embolize the periphery of the second jejunal artery. During the procedure, the prothrombin time was normal, but only the activated partial thromboplastin time was prolonged. A close examination of the coagulation system revealed a decrease in factor VIII levels and the presence of factor VIII inhibitors, resulting in the diagnosis of AHA. Administration of steroids was initiated on PD 15 and, in addition to daily blood transfusions, activated prothrombin complex concentrate was administered to achieve hemostasis. The patient was discharged from the intensive care unit on PD 36 but later developed an intractable labial fistula due to suture failure at the gastrojejunostomy site. As the use of factor VIII inhibitors continued despite the administration of steroids, cyclophosphamide (CPA) pulse therapy was added at PD 58. However, CPA was ineffective and the administration of rituximab was initiated on PD 98. After 12 courses of rituximab, the patient tested negative for factor VIII inhibitors on PD 219. On PD 289, labial fistula closure was performed with continuous replacement of factor VIII and the patient was discharged on PD 342. 

pdf/a-case-of-acquired-hemophilia-a-after-2q2e0lgq45.pdf

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

Define consensus recommendations to improve care coordination between Hospital Pharmacy, Hematology and Nursing, inter- and intra-center, in the care of hemophilia patients. Recommendations for the improvement of care coordination in the management of hemophilia patients were identified and assessed by a multidisciplinary panel of professionals with experience in this field (Hospital Pharmacy, Hematology and Nursing) and supported by scientific evidence. The identified recommendations were assessed by Rand/UCLA consensus methodology (Delphi-adapted) based on their appropriateness and, subsequently, on their necessity. In both cases, it was used ordinal Likert scale. Data were statistically analyzed through different metrics. Fifty-three recommendations for the improvement of care coordination between Hospital Pharmacy, Hematology and Nursing in the management of hemophilia patients were identified, grouped into eight areas of action: i) Hemophilia units, reference centers and multidisciplinary care; ii) Role of Hematology, Hospital Pharmacy and Nursing in the patient journey of hemophilia patients; iii) Telepharmacy and telemedicine; iv) Pharmacokinetic monitoring; v) Transition to adult patient regimen; vi) Patient health education; vii) Surgery, emergency room and hospital admission; and viii) Outcome evaluation. All recommendations were assessed as appropriate and necessary by the external expert panel. Hemophilia patient journey is complex and depends on different variables. It also requires the involvement of different healthcare professionals who must act in a coordinated and integrated manner at all stages of the patient's life, adapted to their individual needs. On this matter, the identified and agreed recommendations may improve continuity and quality of care, as they facilitate the integration and coordination of the professionals involved in the management of this pathology, especially Hospital Pharmacy, Hematology and Nursing. Definir recomendaciones consensuadas para mejorar la coordinación asistencial entre Farmacia Hospitalaria, Hematología y Enfermería, inter e intracentros, en la atención a los pacientes con hemofilia. Se identificaron y valoraron recomendaciones para la mejora de la coordinación asistencial en el abordaje de los pacientes con hemofilia, por parte de un panel multidisciplinar de profesionales con experiencia en este campo (farmacia hospitalaria, hematología y enfermería) y apoyado en la evidencia científica. La valoración de las recomendaciones identificadas se realizó por metodología de consenso Rand/UCLA (Delphi-adaptado) en base a su adecuación y, posteriormente, a su necesidad. En ambos casos, se empleó la escala ordinal de Likert. Los datos se analizaron estadísticamente a través de diferentes métricas. Se identificaron 53 recomendaciones para la mejora de la coordinación asistencial entre farmacia hospitalaria, hematología y enfermería en el manejo del paciente con hemofilia, agrupadas en 8 ámbitos de actuación: i) unidades de hemofilia, centros de referencia y abordaje multidisciplinar; ii) papel de la hematología, la farmacia hospitalaria y enfermería en el recorrido asistencial de los pacientes con hemofilia; iii) telefarmacia y telemedicina; iv) monitorización farmacocinética; v) transición al régimen de paciente adulto; vi) educación sanitaria al paciente; vii) cirugía, urgencias e ingreso hospitalario; y viii) evaluación de los resultados. Todas las recomendaciones fueron valoradas por el panel de expertos externos como adecuadas y necesarias. El recorrido asistencial del paciente con hemofilia es complejo y depende de diversas variables. Además, requiere la implicación de distintos profesionales sanitarios que deben actuar de manera coordinada e integrada en todas las etapas de la vida del paciente, de manera adaptada a sus necesidades individuales. Las recomendaciones identificadas y consensuadas pueden suponer una mejora para la continuidad y calidad asistencial, pues facilitan la integración y coordinación de los profesionales implicados en el abordaje de esta enfermedad, especialmente de farmacia hospitalaria, hematología y enfermería. 

Consensus recommendations for the improvement of inter- and intra-centre care coordination in the management of hemophilia.

Hemophilia A, also known as factor VIII deficiency, is a rare disorder caused by an insufficient level of factor VIII, an essential clotting protein. Hemophilia A can be inherited or acquired. Inherited hemophilia A is caused by a mutation to the factor VIII gene on the X chromosome, which is commonly passed down from parents to children. However, in about one-third of cases, the cause is a spontaneous mutation in that gene. Acquired hemophilia A is due to an autoantibody to factor VIII, which is termed an inhibitor. This rare disorder can cause life-threatening bleeding complications. Management relies on a rapid and accurate diagnosis, control of bleeding episodes, and eradication of the inhibitor by immunosuppression therapy. Most treatment strategies are centered around anecdotal reports or small case series. This case report summarizes the successful treatment of a patient with acquired hemophilia A and major bleeding following a surgical procedure, with the use of desmopressin, recombinant factor VIIa, repeated doses of recombinant factor VIII, rituximab, and prednisone.

Acquired Hemophilia A: A Case Report.

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Acquired Hemophilia A: A Case Report.