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Diagnostics
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Diagnostic and Management of Acquired Hemophilia
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Diagnostic and Management of Acquired Hemophilia

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (31 December 2022) | Viewed by 10096

Special Issue Editor

Prof. Dr. Ezio Zanon

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Guest Editor
Haemophilia Centre, Department of Medicine, University of Padua Medical School, 35128 Padova, Italy
Interests: hemophilia A and B; Von Willebrand disease; rare coagulation disorders; antipholipid antibodies (lupus anticoagulant, anticardiolipin antibodies); deep vein thrombosis and pulmonary embolism; venous thromboembolism; arterial thrombosis
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues, 

Acquired hemophilia A is a rare bleeding disorder caused by a spontaneous development of autoantibodies against the coagulation factor VIII in males and females with a previously normal hemostasis.

Morbidity and mortality associated with AHA are high, especially in elderly patients with severe co-morbidities.

The use of hemostatic agents to control bleeding and immunosuppressive therapy to eliminate the inhibitor are the gold standard of the treatment.

Hemostatic therapy regimens involve recombinant activated factor VII (rVIIa) and/or activated prothrombin concentrate complex (aPCC) and, more recently, recombinant porcine FVIII (rpFVIII), but it is not clear how they should be chosen to obtain the best control of bleeding.

Steroids, cyclophosphamide, or rituximab are used as immunosuppressive therapy; therefore, it would be important to use these drugs alone or combined in different ways depending on the cause of acquired hemophilia.

Laboratory diagnosis can be complicated, and several limitations and pitfalls exist in the assays used to diagnose AHA.

This Special Issue in “Diagnostic and Management of Acquired Hemophilia” aims to provide an overview of current laboratory diagnosis and strategy of treatment.

Full research papers, communications, and review articles are welcome to give answers to the many questions and concerns related to this field.

Dr. Ezio Zanon
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Diagnostics is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • acquired hemophilia A
  • FVIII
  • inhibitor
  • autoantibody
  • assay methods
  • recombinant porcine FVIII
  • recombinant activated FVII
  • activated prothrombin complex concentrate

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Published Papers (2 papers)

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Research

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10 pages, 603 KiB  
Article
Analytical Performance of Different Laboratory Methods for Measuring Susoctocog-Alfa
by Cristina Novembrino, Ilaria Quaglia, Angelo Claudio Molinari, Alessandra Borchiellini, Antonio Coppola, Rita Carlotta Santoro, Massimo Boscolo-Anzoletti, Eleonora Galbiati, Ezio Zanon and Alessandra Valpreda
Diagnostics 2022, 12(8), 1999; https://doi.org/10.3390/diagnostics12081999 - 18 Aug 2022
Cited by 5 | Viewed by 1670
Abstract
Recombinant porcine factor VIII (rpFVIII) is indicated for treating bleeding episodes in acquired haemophilia A, but there are few data regarding laboratory methods to adequately monitor treatment. This study involving three Italian laboratories aimed to evaluate the analytical performance of different assays for [...] Read more.
Recombinant porcine factor VIII (rpFVIII) is indicated for treating bleeding episodes in acquired haemophilia A, but there are few data regarding laboratory methods to adequately monitor treatment. This study involving three Italian laboratories aimed to evaluate the analytical performance of different assays for measuring rpFVIII. Five spiked rpFVIII samples (0.5–1.5 IU/mL) were analysed on three days, in triplicate, with eleven combinations of reagents (Werfen, Boston, MA, USA: SynthasIL and SynthaFax for one-stage assay, Chromogenix Coamatic FVIII for chromogenic assay), FVIII depleted plasmas (with or without von Willebrand factor—VWF) and calibrators (HemosIL human calibrator plasma, porcine calibrator diluted in FVIII deficient plasma with or without VWF). The assays were performed on ACL TOP analysers (Werfen, Boston, MA, USA). Intra- and inter-assay and inter-laboratory Coefficient of Variation (CV%) were calculated together with percentage of recovery (% recovery) on the expected value. The results showed that the reagent combinations reaching satisfactory analytical performance are: SynthasIL/human calibrator/deficient plasma+VWF (total recovery 99.4%, inter-laboratory CV 4.04%), SynthasIL/porcine calibrator/deficient plasma+VWF (total recovery 111%, inter-laboratory CV 2.75%) and Chromogenic/ porcine calibrator/deficient plasma+VWF (total recovery 96.6%, inter-laboratory CV 8.32%). This study highlights that the use of porcine standard (when available) and FVIII deficient plasma with VWF should be recommended. Full article
(This article belongs to the Special Issue Diagnostic and Management of Acquired Hemophilia)
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Review

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19 pages, 774 KiB  
Review
Acquired Hemophilia A: An Update on the Etiopathogenesis, Diagnosis, and Treatment
by Ezio Zanon
Diagnostics 2023, 13(3), 420; https://doi.org/10.3390/diagnostics13030420 - 23 Jan 2023
Cited by 12 | Viewed by 7817
Abstract
Acquired haemophilia A (AHA) is a rare bleeding disorder caused by inhibitory autoantibodies against coagulation factor VIII (FVIII). AHA is a disease that most commonly affects the elderly but has also been observed in children and in the postpartum period. AHA is idiopathic [...] Read more.
Acquired haemophilia A (AHA) is a rare bleeding disorder caused by inhibitory autoantibodies against coagulation factor VIII (FVIII). AHA is a disease that most commonly affects the elderly but has also been observed in children and in the postpartum period. AHA is idiopathic in 50% of cases and is associated with autoimmune diseases, malignancies, and infections in the remaining 50%. Recently, cases of association between AHA, COVID-19 vaccination, and infection have been reported in the literature. For diagnoses, determining FVIII levels is crucial to distinguish the different causes of aPTT prolongation. Treatment of AHA is based on bypassing agents (recombinant factor VIIa, activated prothrombin complex concentrate) and porcine FVIII to control the bleeding and immunosuppressive therapy (corticosteroids, rituximab, cyclophosphamide) to suppress autoantibody production. It is important to start a prophylactic regimen to prevent further bleeding episodes until the inhibitor is negative. Recently, the series of cases reported in the literature suggest that emicizumab may provide effective and safe haemorrhage prophylaxis in the outpatient setting. Full article
(This article belongs to the Special Issue Diagnostic and Management of Acquired Hemophilia)
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