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Acquired haemophilia A: successful treatment of a patient using upfront immunosuppressive therapy and haemostatic agents
  1. Su Yun Chung1,2,
  2. Janice Gloria Shen1,3 and
  3. Kristin Lynn Sticco4,5
  1. 1Monter Cancer Center, Long Island Jewish Medical Center Northwell Health Cancer Institute, Lake Success, New York, USA
  2. 2Division of Hematology and Medical Oncology, Department of Medicine, Northwell Health, New Hyde Park, New York, USA
  3. 3Division of Hematology and Medical Oncology, Department of Medicine, Northwell Health, Manhasset, New York, USA
  4. 4Pathology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York, USA
  5. 5Department of Anatomic and Clinical Pathology, Northwell Health, Greenvale, New York, USA
  1. Correspondence to Dr Kristin Lynn Sticco; ksticco@northwell.edu

Abstract

Acquired haemophilia A (AHA) is a rare and possibly fatal autoimmune disorder that is challenging to treat. Although a majority of cases are idiopathic, AHA can also be associated with an underlying malignancy, autoimmune disorder, pregnancy, infection or certain medications. The diagnosis and treatment of AHA require a specialist with both clinical and laboratory expertise. The goal of treatment is aimed at achieving haemostasis as well as eradicating factor inhibitors. We present a patient with AHA and life-threatening haemorrhage who was successfully treated with a combination of haemostatic agents and a triple-drug immunosuppressive regimen. In reviewing recent studies and published guidelines, we advocate that a newer agent, emicizumab, can potentially be incorporated into the treatment protocol for AHA given its promising performance in the realm of congenital haemophilia.

  • haematology (incl blood transfusion)
  • malignant and benign haematology

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Footnotes

  • Twitter @ksticcoMD

  • Contributors SYC designed the concept of the case report and worked on the image files. JGS and KLS gathered and organised most of the references. All authors equally contributed to writing the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.