Spontaneous Cervical Spinal Epidural Hematoma in Myelofibrosis

Introduction: Spontaneous spinal epidural hematomas (SSEHs) are well-documented with an incidence of 0.1 per 100,000 individuals. Their occurrence with myelofibrosis, a myeloproliferative neoplasm (MPN), is exceedingly uncommon. Treatment routinely involves kinase inhibitors, such as ruxolitinib, known to cause thrombocytopenia. We present a rare case of a patient with myelofibrosis and thrombocytopenia who developed a cervical SSEH.


Description: A 69 year-old woman with myelofibrosis, treated with ruxolitinib, was admitted for hematopoietic cell transplantation. Post-transplant, she developed acute neck pain, dyspnea, and hypertension followed by new left hemiplegia and right-sided weakness. CT head was unremarkable but, post-imaging, she developed worsened right-sided weakness and sensory loss at the T2-T3 level. This prompted a CT spine, which revealed fullness of the cervical epidural space with associated cord compression. Her work of breathing then worsened, requiring intubation and intensive care unit (ICU) care. She was deemed not a surgical candidate due to thrombocytopenia and was managed with steroids, platelet transfusions, and antihypertensives. Later, MRI confirmed cord compression by SSEH at the C2 to T2/T4 levels. Her neurologic status gradually improved with conservative management, and she was extubated 2 days later.


Discussion: Cervical SSEHs can result in devastating consequences such as quadriplegia or Brown-Sequard syndrome, characterized by ipsilateral motor and sensory deficits. Risk factors include bleeding disorders and hypertension. Intraspinal vessel rupture is a suspected etiology, and patients typically present with acute pain and radicular symptoms around the involved vertebrae. Neurologic deficits include weakness, paresis, bladder dysfunction, and sensory deficit. MRI is the preferred diagnostic tool and treatment of choice is decompressive surgery. Mortality affects one-third of cases, most commonly due to respiratory failure. In this patient, hypertension likely precipitated an intraspinal vascular rupture, resulting in SSEH which expanded rapidly due to thrombocytopenia. This case emphasizes the value of increased vigilance for SSEH among patients with MPN, commonly managed with thrombocytopenia-inducing therapies, and concurrent hypertension.