gms | German Medical Science

14th Triennial Congress of the International Federation of Societies for Surgery of the Hand (IFSSH), 11th Triennial Congress of the International Federation of Societies for Hand Therapy (IFSHT)

17.06. - 21.06.2019, Berlin

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Acquired hemophilia A as a rare cause of nontraumatic compartment syndrome and hematoma formation

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  • presenting/speaker Daniel Walder - Kantionsspital Aarau, Aarau, Switzerland
  • Flavien Mauler - Kantionsspital Aarau, Aarau, Switzerland
  • Claudia Meuli - Kantionsspital Aarau, Aarau, Switzerland
  • Thuan Ly - Kantionsspital Aarau, Aarau, Switzerland

International Federation of Societies for Surgery of the Hand. International Federation of Societies for Hand Therapy. 14th Triennial Congress of the International Federation of Societies for Surgery of the Hand (IFSSH), 11th Triennial Congress of the International Federation of Societies for Hand Therapy (IFSHT), 11th Triennial Congress of the International Federation of Societies for Hand Therapy (IFSHT). Berlin, 17.-21.06.2019. Düsseldorf: German Medical Science GMS Publishing House; 2020. DocIFSSH19-1621

doi: 10.3205/19ifssh0169, urn:nbn:de:0183-19ifssh01691

Published: February 6, 2020

© 2020 Walder et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

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Objectives/Interrogation: Acquired factor VIII inhibitor (hemophilia A) is a very rare, often idiopathic disease that can lead to diffuse bleeding, with a high rate of morbidity and mortality. We present two patients with first-time diagnosed acquired hemophilia A who were treated at our clinic within 2 months.

Methods: The first patient was an 83-year-old woman who presented with atraumatic compartment syndrome of the right hand which required fasciotomy. The compartment syndrome was due to spontaneous bleeding. Only after several revision surgeries and a complicated course, the resulting defects could be closed by secondary sutures and skin grafting.

The second patient was an 85-year-old man who was admitted with multiple hematomas on both upper extremities, which were treated nonoperatively. The two patients received interdisciplinary care, involving early initiation of clotting factor replacement and immunosuppressive therapy, which were necessary to control the bleeding.

Results and Conclusions: Acquired hemophilia A must be considered as a very rare but important life-threating differential diagnosis in multiple hematomas, especially in atraumatic compartment syndrome.

Early diagnosis with immediate start of replacement therapy to correct coagulation and initialization of immunosuppressive therapy are crucial for successful treatment.