Spontaneous Spinal Epidural Hematoma as the Initial Presentation of Polycythemia Vera

Abstract

relevant medical problems. On admission, her vital signs, cardiovascular and respiratory systems were all unremarkable. Her abdomen was soft and nontender, but an enlarged spleen was palpated 5 cm below the left costal margin. Initial neurological examination was reported as normal. Her blood tests revealed a hemoglobin of 18.1 g/dl, a hematocrit of 55.7% and platelet levels of 810 ! 10 9 /l. White blood cells were 24 ! 10 9 /l (93% neutrophils, 3.7% monocytes, 3% lymphocytes, 0.2% eosinophils, 0.1% basophils) and red blood cells were 6.95 ! 10 12 /l. Her partial thromboplastin time was on the upper end of normal at 37 s (normal 25–37) but her prothombin time was within normal limits at 89 s (normal 75–135). A computed tomography scan of the lumbar spine demonstrated normal anatomical alignment of her vertebrae and discs, but an enlarged spleen was evident along with a dilated splenic artery. After 2 h the patient still experienced unremitting back pain and started to have difficulty moving her legs. Over the following 2 h this developed into a complete flaccid paraplegia with a T10 sensory level and with an atonic anal sphincter. Urgent magnetic resonance imaging of the thoracic-lumbar region was performed, revealing a heterogenous, posterior thoracic epidural lesion at the T7–T12 level causing spinal cord compression. The appearance was consistent with an acute epidural hePolycythemia vera (PV) is a myeloproliferative disorder characterized by clonal proliferation of hematopoietic stem cells leading to the accumulation of erythrocytes, leukocytes, and platelets in the circulation [1] . Patients with PV and other myeloproliferative disorders often suffer from thrombotic and, less commonly, hemorrhagic complications. We describe the unique case of a patient with an undiagnosed myeloproliferative disorder who presented with acute back pain and an abnormal full blood count. She had initially no apparent neurological deficit but her symptoms rapidly regressed to complete paraplegia. She was found to have had a spontaneous spinal epidural hematoma of the thoracic spine. A 66-year-old woman arrived at our Emergency Department with a 3-hour history of severe back pain; the pain originated from her thoracic spine, was intense in nature and radiated to both her lower limbs. There was no history of direct trauma or change in her physical activities. She denied any pyrexia, illness, bruising or bleeding in the period prior to her presentation. Her comorbidities consisted only of peptic ulcer disease for which she underwent gastrectomy 20 years previously. She also had a 1-year history of a high hemoglobin level for which there had been no further investigations. She had been taking 100 mg of aspirin daily for 1 year as a primary preventive measure. She was a nonsmoker and had no other Received: August 27, 2009 Accepted after revision: September 23, 2009 Published online: November 21, 2009