Abstract

IntroductionIliopsoas hemorrhage is a serious complication of bleeding disorders that occurs most commonly in patients with hemophilia and less commonly in patients with von Willebrand disease. It causes severe pain, muscle dysfunction and occasionally femoral nerve palsy. We describe the case of a patient with von Willebrand disease type 3 with a large iliopsoas hematoma who was treated with a von Willebrand factor concentrate (Humate-P).Case presentationA 20-year-old Iranian man was referred to our emergency ward because of the gradual onset of right flank pain. He was known to have been diagnosed with von Willebrand disease type 3 at age two years old. Magnetic resonance imaging showed a mass in the right iliopsoas muscle. The diagnosis of iliopsoas hemorrhage and partial femoral nerve palsy was established, and he responded to medical treatment.ConclusionWe report a case of von Willebrand disease type 3 with spontaneous iliopsoas hematoma associated with femoral nerve palsy that was well managed with Humate-P treatment.

Highlights

  • Iliopsoas hemorrhage is a serious complication of bleeding disorders that occurs most commonly in patients with hemophilia and less commonly in patients with von Willebrand disease

  • We report a case of von Willebrand disease type 3 with spontaneous iliopsoas hematoma associated with femoral nerve palsy that was well managed with Humate-P treatment

  • Iliopsoas hemorrhage, whether spontaneous or traumatic, is encountered in a variety of coagulation disorders, such as hemophilia and Von Willebrand disease (vWD), and in association with anti-coagulant drug treatments such as heparin, warfarin and ticlopidine; most cases of iliopsoas hemorrhage occur in patients with hemophilia [3], and the first case was reported by Tallroth in 1939 [4]

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Summary

Introduction

Von Willebrand disease (vWD) is a hereditary bleeding disorder caused by a quantitative or qualitative defect of von Willebrand factor. He was known to have had vWD type 3 since he was two years old His laboratory findings were as follows: B-positive blood type, partial thromboplastin time 56 seconds, bleeding time more than 12 minutes, hemoglobin 10 g/dL, mean corpuscular volume 75 fL, mean corpuscular hemoglobin 24 pg/ red blood cell, factor VIIIc activity 3%, vWF antigen level was undetectable, and vWF ristocetin cofactor activity was undetectable. He had experienced some occasional hemarthrosis, but he had been well for two months before he presented to our hospital. His follow-up physical examination showed a healthy quadriceps muscle function, a good patellar tendon reflex and an intact sensory femoral nerve

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Mannucci PM
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