Introduction: We present a case of spontaneous compartment syndrome due to a very rare cause which is acquired hemophilia.

Case Presentation: A 34-year-old patient presented with the right thigh swelling and features of acute compartment syndrome without history of trauma. He had no history of bleeding disorder. There were no features of infection. As his initial blood tests were within normal 16 g/dl, and his compartment syndrome worsened, he had decompression of the thigh. During the post-operative period, the patient developed persistent bleeding from the decompressed wounds and a fall in hemoglobin which led to further investigation when the blood profile showed a deficiency of factor VIII and antibodies to factor VIII, diagnosis of acquired hemophilia was made and appropriate treatment given.

Conclusion: If atraumatic compartment syndrome diagnosed, possibility of acquired factor VIII deficiency should be raised by isolated prolonged activated partial thromboplastin time and diagnosis confirmed by measuring factor VIII activity level and detecting any factor's VIII antibodies in blood, such as in this case, the factor VIII level was 5.5 (very low) and against factor VIII antibodies level was 60.8 (high). Here, hematologist should be involved in management.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8686512PMC
http://dx.doi.org/10.13107/jocr.2021.v11.i08.2366DOI Listing

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