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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 54  |  Issue : 5  |  Page : 200-202

Spontaneous spinal epidural hemorrhage due to polycythemia vera


1 Department of Surgery, Division of Neurosurgery, Mackay Memorial Hospital, Taipei, Taiwan
2 Department of Emergency Medicine, Mackay Memorial Hospital, Taipei, Taiwan
3 Division of Neurology, Yunlin Chang-Gung Memorial Hospital, Yunlin, Taiwan
4 Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan

Date of Submission09-May-2021
Date of Decision17-Jun-2021
Date of Acceptance07-Sep-2021
Date of Web Publication12-Oct-2021

Correspondence Address:
Hsin-Yao Lin
No. 92, Sec. 2, Zhongshan North Road, Taipei 10449
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_92_21

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  Abstract 


Spontaneous spinal epidural hematoma (SSEH) as an initial presentation of polycythemia vera (PV) is rare. It can present with the features ranging from simple back pain to complete paraplegia depending on the site of the spinal compression. Herein, we describe a case of undiagnosed PV presented as back pain with radiation to the chest wall in a 46-year-old male patient and subsequently diagnosed with spontaneous spinal epidural hemorrhage. To the best of our knowledge, only one case of spinal epidural hematoma-related PV has been reported so far. Emergent decompressive laminectomy was done within 10 h of initial presentation with excellent clinical outcome. Therefore, this condition must be promptly diagnosed and treated with decompression surgery to minimize the risk of neurological complication.

Keywords: Decompressive laminectomy, polycythemia vera, spontaneous spinal epidural hematoma


How to cite this article:
Lin TY, Chien SC, Chu CL, Jhuang JY, Tsai CC, Lin HY. Spontaneous spinal epidural hemorrhage due to polycythemia vera. Formos J Surg 2021;54:200-2

How to cite this URL:
Lin TY, Chien SC, Chu CL, Jhuang JY, Tsai CC, Lin HY. Spontaneous spinal epidural hemorrhage due to polycythemia vera. Formos J Surg [serial online] 2021 [cited 2021 Nov 28];54:200-2. Available from: https://www.e-fjs.org/text.asp?2021/54/5/200/327889




  Introduction Top


The incidence of spontaneous spinal epidural hematoma (SSEH) has been estimated at 0.1 patients per 100,000 persons and represents <1% of spinal space-occupying lesions.[1] Polycythemia vera (PV) is a rare type of myeloproliferative neoplasm in which the bone marrow overproduces red blood cells. It has various clinical presentations such as headache, excessive sweating, blurred vision, thrombosis, hemorrhage, hypertension, palpable spleen, and vasomotor symptoms.[2] Early diagnosis of PV is important in emergency medicine as it poses a risk of severe morbidities such as myocardial infarction, ischemic stroke, cardiac tamponade, spontaneous omental bleeding, or ventricular fibrillation.[2] In this report, we describe a rare case of PV accompanied by SSEH in a 46-year-old male who presented with acute paraparesis with back pain and underwent decompressive laminectomy with complete recovery.


  Case Report Top


A 46-year-old man was found to have a sudden onset of progressive right chest pain to upper back pain 12 h prior to emergency department visit. The patient denied any history of trauma and had no medical history. Upon arrival at the emergency department, the patient was alert, orientated, and afebrile with blood pressure of 176/131 mmHg and heart rate of 124 beats per minute. The laboratory data indicated profound leukocytosis with polycythemia (white blood cell count: 20,300/μL, hemoglobin: 20.7 g/dL, hematocrit [Hct]: 58.7%, and neutrophil percentage: 89.2%); renal function impairment (blood urine nitrogen: 84.2 mg/dL and creatinine: 4.79 mg/dL); and coagulopathy (prothrombin time and activated prothrombin time increased 1.4-fold). Chest and abdominal computed tomography with and without contrast revealed suspicious hepatosplenomegaly with subcentimeter lymph nodes in the upper para-aortic region. The hematologist was consulted and PV was suspected. There was no evidence of aortic dissection or pulmonary thromboembolism.

After 9 h in the emergency department, the patient presented with urinary retention, bilateral lower-limb numbness, and weakness (muscle power grade 3 out of 5) with hyporeflexia on neurological examination. A sensory deficit with loss of pinprick sensation was confirmed below T10 level despite a normal joint position and response to vibration. The American Spinal Injury Association (ASIA) score is D. Magnetic resonance imaging (MRI) of the thoracic and lumbar spinal cord revealed a posterior epidural lesion involving the C7–T7, with diffusion restriction, causing the compression of the thecal sac and spinal cord. Differential diagnosis included epidural abscess or hematoma [Figure 1]. Thus, an emergent total laminectomy from T1 to T6 was performed to remove the epidural lesion. Pathological reports indicated a focal hemorrhage with no evidence of malignancy or infection.
Figure 1: Magnetic resonance imaging of the thoracic spine. A posterior heterogeneous thoracic epidural lesion (arrows) at the C7 ~ T7 level with spinal cord compression. (a) Sagittal view, T2 sequence. (b) Sagittal view, T1 sequence. (c) Axial view, T2 sequence

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On postoperative day 2, the patient's muscle power in both legs improved. The genetic test revealed point mutation V617F in JAK2 exon 14. Bone marrow biopsy showed hypercellular marrow for his age with panmyelosis (proliferation of three lineages, including erythroid series, myeloid series, and megakaryocytes) (H and E stain, ×400). Immunostains myeloperoxidase and E-cadherin helped highlighting the extent of myeloid and erythroid series. CD34 stain showed no excess blasts (<2%) [Figure 2]. According to clinicopathological features, the diagnosis was PV. Repeated therapeutic phlebotomy was performed during hospitalization. After discharge, the patient was able to walk without any assistant device and regularly followed up in outpatient department.
Figure 2: Bone marrow biopsy. (a) Hypercellular marrow with panmyelosis and no excess blasts, H and E, ×400. (b) CD34 ×400 NO excess blasts (<2%). (c) MPO ×400 proliferation of myeloid. (d) E-cadherin ×400 proliferation of erythroid series

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  Discussion Top


SSEH is a rare critical disease with spinal cord compression causing devastating neurological deficits. Moreover, its pathogenesis is not fully understood. Previous studies suggested that SSEH can be induced by ruptured epidural vein, epidural artery, or vascular malformation.[3] The epidural plexus is a low pressure and valveless system that may rupture when pressure increased.[4]

Blood viscosity is an intrinsic property of fluid related to the internal friction of adjacent fluid layers sliding past one another.[5] Factors determining the viscosity of blood are erythrocytes, leukocyte masses, plasma fibrinogen, proteins, and deformability of erythrocytes.[5],[6] The relationships between blood viscosity, Hct, and blood pressure are in direct proportion.[7] The sustained hyperviscosity can decrease tissue perfusion and increase blood pressure. Meanwhile, it may increase shear stress on the endothelium and nitric oxide release, promoting vasodilation and prone to vessel rupture.[7] The above theory supports that the cause of SSEH is related to hyperviscosity and hypertension due to PV.

In our review of literature, there was only one report by Kivity et al. in 2010 of a case of SSEH in an undiagnosed PV treated with aspirin.[8] Aspirin and underlying coagulopathy are considered precipitating factors. Early diagnosis of PV and awareness of the trigger factors of SSEH are crucial for timely diagnosis and management.

The typical symptoms of SSEH are sudden severe neck or back pain accompanied by paralysis of the extremities, bladder, and bowels. However, the onset of neurological deficits may be delayed after back pain from several hours to months.[3] Once SSEH is impressed, MRI is the gold standard diagnostic imaging technique. Raasck et al. have shown that over 50% of patients continue to have some level of sensorimotor deficiency in ASIA score of A through D posttreatment.[9] Shin et al. have described that the recovery scale of the Japanese Orthopaedic Association was 84%, 64%, and 47% for the SSEH patients who received operation after the onset of symptoms <12 h, between 12 and 24 h, and after 24 h, respectively.[10] In our case, the clinical presentation was typical, and the interval between back pain and the neurological deficit was 9 h. In the emergency department, rapid MRI was performed and surgical intervention was done immediately. Subsequently, the patient achieved full recovery.

For treatment, decompressive laminectomy with hematoma evacuation is the standard treatment for patients with SSEH presenting with severe neurological deficits. Conservative treatment is an option in case of relatively subtle neurological deficits in which the patient would need to undergo serial neurologic examination and bed rest. Due to the urgency of the disease, spinal cord function should be checked immediately and decompressive surgery should be arranged as soon as possible.


  Conclusion Top


SSEH as a potential complication of PV is rare but should be kept aware of in the emergency department. The pathophysiology of SSEH may result from hyperviscosity, hypertension, and increased shear force. Early MRI should be performed timely for differential diagnosis of thrombotic or hemorrhagic events. Early decompressive surgery prior to total paralysis is recommended for patients presenting with SSEH to achieve good postoperative functional outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Taniguchi LU, Pahl FH, Lúcio JE, Brock RS, Gomes MQ, Adoni T, et al. Complete motor recovery after acute paraparesis caused by spontaneous spinal epidural hematoma: Case report. BMC Emerg Med 2011;11:10.  Back to cited text no. 1
    
2.
Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: An international study. Leukemia 2013;27:1874-81.  Back to cited text no. 2
    
3.
Figueroa J, DeVine JG. Spontaneous spinal epidural hematoma: Literature review. J Spine Surg 2017;3:58-63.  Back to cited text no. 3
    
4.
Zhong W, Chen H, You C, Li J, Liu Y, Huang S. Spontaneous spinal epidural hematoma. J Clin Neurosci 2011;18:1490-4.  Back to cited text no. 4
    
5.
Yarnell JW, Baker IA, Sweetnam PM, Bainton D, O'Brien JR, Whitehead PJ, et al. Fibrinogen, viscosity, and white blood cell count are major risk factors for ischemic heart disease. The caerphilly and speedwell collaborative heart disease studies. Circulation 1991;83:836-44.  Back to cited text no. 5
    
6.
Vázquez BY. Blood pressure and blood viscosity are not correlated in normal healthy subjects. Vasc Health Risk Manag 2012;8:1-6.  Back to cited text no. 6
    
7.
Cinar Y, Demir G, Paç M, Cinar AB. Effect of hematocrit on blood pressure via hyperviscosity. Am J Hypertens 1999;12:739-43.  Back to cited text no. 7
    
8.
Kivity S, Rajz G, Segal G, Merkel D, Sidi Y. Spontaneous spinal epidural hematoma as the initial presentation of polycythemia vera. Acta Haematol 2010;123:34-6.  Back to cited text no. 8
    
9.
Raasck K, Habis AA, Aoude A, Simões L, Barros F, Reindl R, et al. Spontaneous spinal epidural hematoma management: A case series and literature review. Spinal Cord Ser Cases 2017;3:16043.  Back to cited text no. 9
    
10.
Shin JJ, Kuh SU, Cho YE. Surgical management of spontaneous spinal epidural hematoma. Eur Spine J 2006;15:998-1004.  Back to cited text no. 10
    


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