Keikhaei and Soltani Shirazi Journal of Medical Case Reports 2011, 5:274 http://www.jmedicalcasereports.com/content/5/1/274 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Spontaneous iliopsoas muscle hematoma in a patient with von Willebrand disease: a case report Bijan Keikhaei1* and Ahmad Soltani Shirazi2 Abstract Introduction: 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. 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 presentation: A 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. Conclusion: 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. Introduction Von Willebrand disease (vWD) is a hereditary bleeding disorder caused by a quantitative or qualitative defect of von Willebrand factor. The prevalence of this disease is estimated to be 1% to 2% of the general population. Von Willebrand factor is a coagulation factor for initial stages of blood clotting and is also a carrier for factor VIII. Patients with vWD develop coagulation platelet plugs and red coagulation clots are unable to form. vWD consists of a broad variation of clinical manifesta- tions, with one extreme being a minimal bleeding ten- dency and the other being a severe presentation such as severe hemophilia A. Luckily, most of these patients present with mild to moderate clinical symptoms. The severe type of the vWD, type 3, occurs in less than 5% of patients. The inheritance pattern of this type is usually autosomal recessive. Patients with vWD usually show clinical features such as recurrent nosebleeds, easy bruising, bleeding post-tooth extraction, post- * Correspondence: keikhaeib@yahoo.com 1Department of Thalassemia & Hemoglobinopathy Research Center, Ahvaz Jundishapur University of Medical Science, Shafa Hospital (Golestan Area), Ahvaz 61357-33119, Iran Full list of author information is available at the end of the article tonsillectomy or other surgical bleeding, and excessive menstrual bleeding. Musculoskeletal hemorrhage occurs commonly in vWD type 3-like hemophilia A. Iliopsoas hemorrhage is a potentially serious complication because a delay in beginning treatment may result in permanent femoral nerve palsy [1]. The present case report describes a man with vWD type 3 who had a spontaneous iliopsoas hematoma that responded well to Humate-P treatment (CSL Behring, King of Prussia, PA, USA) [2]. Case presentation A 20-year-old Iranian man came to our hospital because of the gradual onset of right flank pain. 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 corpus- cular 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 hemar- throsis, but he had been well for two months before he presented to our hospital. He had no history of trauma. © 2011 Keikhaei and Shirazi; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.