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Table of Contents
Year : 2021  |  Volume : 17  |  Issue : 1  |  Page : 21-24

Cerebral venous sinus thrombosis as an uncommon presentation of polycythemia vera

1 Department of General Medicine, Command Hospital (Western Command), Panchkula, Haryana, India
2 Department of Radiology, Command Hospital (Western Command), Panchkula, Haryana, India

Date of Submission25-Dec-2020
Date of Acceptance06-Feb-2021
Date of Web Publication18-May-2021

Correspondence Address:
Dr. Rajeev Saxena
Command Hospital (Western Command), Panchkula, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/amjm.amjm_75_20

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Cerebral venous sinus thrombosis (CVST) accounts for <1% of all cerebrovascular accidents which were attributed to increased viscosity of blood and activation of platelet in blood vessels. Polycythemia vera (PV) increases the risk of vaso-occlusive events including CVST which may be initial unusual presentation of disease. We present a 28-year-old male patient with headache associated with nausea, vomiting, and blurring of vision. Initial clinical examination was unremarkable, and blood investigations revealed erythrocytosis and neutrophilic leukocytosis with prominent granules. Brain imaging studies were consistent of cerebral venous thrombosis with hemorrhagic transformation and perilesional edema with midline shift. He was managed by decompressive hemicraniectomy, low molecular weight heparin, central nervous system decongestants, antiepileptics, and supportive care with close monitoring. PV is a hypercoagulable state which may precipitate vascular thrombosis. CVST is rare presentation of PV. Early screening for occult prothrombotic entities with prompt management increases the patient outcome and prognosis.

Keywords: Cerebral venous sinus thrombosis, erythrocytosis, hypercoagulable state, polycythemia vera

How to cite this article:
Yadav MK, Saxena R, Nagarajan H, Godara CB. Cerebral venous sinus thrombosis as an uncommon presentation of polycythemia vera. Amrita J Med 2021;17:21-4

How to cite this URL:
Yadav MK, Saxena R, Nagarajan H, Godara CB. Cerebral venous sinus thrombosis as an uncommon presentation of polycythemia vera. Amrita J Med [serial online] 2021 [cited 2021 Sep 27];17:21-4. Available from: https://www.ajmonline.org.in/text.asp?2021/17/1/21/316322

  Introduction Top

Cerebrovascular accidents comprise both arterial and venous stroke. Cerebral venous thrombosis (CVT) is a venous stroke comprising thrombosis of central intracranial venous sinuses and cortical veins with varied and often dramatic clinical presentation. It often affects young to middle-aged individuals and more commonly women. CVT accounts for 0.5%–1% of all strokes and 10%–20% of strokes in young.[1] CVT most commonly involves superior sagittal sinus (72%) followed by lateral sinus (70%).[2],[3] CVT has wide spectrum of symptoms and clinical course which varies from headache to focal deficits such as hemiparesis, hemi sensory disturbance, seizures, impairment of level of consciousness and papilledema. All individuals with unusual and intractable headache should be evaluated for CVT. Various etiological factors are attributed to CVT which requires high index of suspicion because of heterogeneity in clinical presentation and etiology. Bacterial meningitis, infection of ear, and paranasal sinuses may lead to septic CVT, while head injury, tumors, oral contraceptive pill, pregnancy, and coagulopathies lead to aseptic CVT.[4] Polycythemia vera (PV) is a chronic clonal stem cell disorder characterized by increased red blood cells, and 50%–80% of the patients have neurological problems.[5] The signs and symptoms of PV are mainly caused by increased blood volume, slowing of blood flow, and thrombotic hemorrhagic complications as a result of increased viscosity. Cerebral venous sinus thrombosis (CVST) is a rare and life-threatening condition that has been associated with various hypercoagulable states, such as thrombocytosis, remote infections, and PV. Here, we present a case of PV presenting as CVT.

  Case Report Top

A 28-year-old gentleman, right-handed, resident of Leh (India), presented with complaints of sudden-onset headache of 6 days' duration. It was associated with nausea, vomiting, and blurring of vision. He denied any weakness of limbs, difficulty in swallowing, or slurred speech and there was no preceding history of any lapses of consciousness or seizure disorder. He was a nonsmoker, nonalcoholic, normotensive, having mixed dietary intake, and denied any illicit drug use. Family history was unremarkable for any neurologic or hematologic disease. On initial clinical examination, pulse was 80 beats/min, regular, and of normal volume. The blood pressure was 130/80 mm of Hg in the right arm in supine position, conjunctivae congested, Glasgow Coma Scale (GCS) was 15/15, pupils were bilaterally normally reactive, and no focal neurological deficit was noted. No other significant abnormality was detected. The clinical examination of other systems was normal. Investigations revealed hemoglobin of 18 g% with hematocrit of 63.5%, total leukocyte count of 10,700/cumm, platelet count of 199,000/cumm, and total red blood cell count of 9,830,000/cumm, with prothrombin time of 14 s with control time of 13 s. His fasting sugars, blood urea, serum creatinine, lipid profile, serum electrolytes, and liver function test were within normal limits. Triple viral screening for HIV, HBsAg, and anti HCV was negative. Peripheral blood smear revealed erythrocytosis and neutrophilic leukocytosis with prominent granules and no blast was seen. D-dimer and fibrin degradation products were increased. On further etiologcal work up, ANA AntidsDNA, CD55 and CD59 (PNH), antiphospholipid antibodies and anticardiolipin antibodies were negative. Hemoglobin pattern was normal on Hb electrophoresis and sickling test was negative. Serum erythropoietin level was <1 IU/L (5-35 IU/L). Echocardiography (ECG) and three-dimensional ECG were normal. Fundoscopic examination revealed bilateral papilledema, and no signs of vitreous hemorrhage and periphery could not be seen due to nondilated pupils. Neuroimaging with computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance (MR) venography revealed CVT involving superior sagittal sinus, straight sinus, torcula, and right transverse sinus causing hemorrhagic infarcts involving the right temporal and parietal lobes with associated perilesional edema and mild midline shift toward left [Figure 1] and [Figure 2]. Contrast-enhanced CT (CECT) of the abdomen and color Doppler of splenoportal axis revealed splenic infract [Figure 3]. With high level of hemoglobin, elevated hematocrit, erythrocytosis, polymorpho-nuclear leukocytosis, radiological evidence of CVST, splenic infract, and low erythropoietin levels, this case was diagnosed as PV with CVST and perilesional edema with splenic infraction. He was managed with injection enoxaparin 60 mg SC BD, injection mannitol 150 ml IV TDS, tablet diamox 500 mg TDS, injection sodium dilantin 20 mg/kg loading dose followed by 300 mg IV HS, phlebotomy, and supportive care with close monitoring. Headache persisted, and on day 5, he developed slurring of speech, drowsiness, weakness in bilateral upper limbs, and altered sensorium. Examination revealed low GCS (E3V3M5) with anisocoria. On investigation thrombocytopenia was present, CT head revealed increased edema with midline shift of 6 mm, new hemorrhagic bleed in left parietal region. Injection low molecular weight heparin was withheld in view of thrombocytopenia. His condition further deteriorated and he was intubated and put on mechanical ventilation. Repeat CT head revealed multiple cortical venous thrombosis with 13 mm midline shift and right uncal herniation. Right decompressive hemicraniectomy was done due to increasing perilesional edema causing midline shift and uncal herniation with progressive decline in GCS. Intra-op brain was tense, nonpulsatile, temporal lobe hematoma with diffuse ooze present. Postoperatively, he was managed with broad-spectrum parental antibiotics, injection noradrenaline, cerebral decongestants, and supportive care. On postoperative day 4, his condition improved, GCS was 15/15, power started improving, inotropes were tapered, he was extubated, platelet increased, and he was started on fondaparinux. Repeat CT head showed improving status with partial regression in perilesional edema and midline shift.
Figure 1: Noncontrast computed tomography brain axial sections (a and b) and coronal sections (c and d) showing hyperdensities involving the superior sagittal sinus, right transverse sinus with hemorrhagic infarcts involving right temporal and parietal lobes and associated perilesional edema and mild midline shift toward left

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Figure 2: Magnetic resonance venogram (a and b) with three-dimensional reconstruction format showing irregularities involving superior sagittal sinus and right transverse sinus

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Figure 3: Contrast-enhanced computed tomography abdomen with coronal (a) and axial (b) sections showing geographic hypoenhancing areas within spleen

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The patient had satisfactory response to the treatment, Neurologic symptoms improved, there were no episodes of seizures, his headache subsided, and he was comfortable over the course in the hospital. He was referred for outpatient hematologic and neurologic follow-ups and to date is doing quite well.

  Discussion Top

PV is a myeloproliferative disorder characterized by elevated production of red blood cell, granulocytes, and megakaryocytes in the absence of a recognizable physiologic stimulus. PV may predispose to occurrence of arterial and venous thrombosis both, but later is less frequent. Defective primary hemostasis occurring in PV leads to various bleeding manifestations in skin and mucous membranes including ecchymosis, epistaxis, menorrhagia, and gingival hemorrhage.[6] PV may affect persons of any age but occurs most commonly in 50–70 years of age.[7] PV has no sex predilection, although the PV study group found that slightly more males than females are affected.[7] Increased blood viscosity in PV is mainly determined by elevated hematocrit. Cerebral blood flow decreases by increased blood viscosity. Platelet marginalization with increased contact to vessel walls occurs, along with local effect of a high hematocrit on vessel walls.[8] This fulfills all three components of Virchow's triad, which leads to arterial and venous thrombosis.[9]

CVST is an uncommon clinical entity which may be septic and aseptic. Infections of ear, paranasal sinuses, and bacterial meningitis may lead to the development of septic CVST. Aseptic CVST etiologically attributed to head injuries, oral contraceptives pills, pregnancy, tumors, and coagulopathies. Sagittal sinus thrombosis (71%) is common because of its high position, low pressure, and slow flow than any other dural sinuses.[4] The symptoms and signs are minimal in occlusion of anterior part of the sagittal sinus such as headache and paresthesia as compared to severe neurological consequences in the posterior part of sagittal sinus. Headache is often worse at night or when arising and may be increased by bending or performing the Valsalva maneuver. Other symptoms such as transiently obscured blurring of vision, diplopia, tinnitus, and syncope spells may also occur. Usually, papilledema is bilateral and symmetric, but it may be unilateral and asymmetric. In PV, papilledema occurs due to raised intracranial pressure from thrombo-occlusion of the superior sagittal sinus, resulting in impaired cerebrospinal fluid absorption. CVT is diagnosed by CT or MRI radiologically. Thrombosis is visualized as linear area of hyperdensity on noncontrast CT scan and as an “empty delta sign” which is a dark triangle in the sinuses where flow is blocked on CECT imaging. Acute thrombosis with absent blood flow in affected sinus may be demonstrated on MRI and MR venography, which is gold standard diagnostic tool in case of a clinical suspicion of thrombosis.

Management of septic CVST should include broad-spectrum antibiotics, treatment of seizures, detection and management of metabolic derangements, and management of cerebral edema and elevated intracranial pressure. Anticoagulants are used to combat risk of hemorrhage in aseptic thrombosis. Patient with preidentified pro-thrombotic state or refractory cases will require prolonged anticoagulation. The goal of management in CVST patient with PV is to maintain normal hematocrit level and reduce high blood viscosity by doing periodic phlebotomies, thus preventing further thrombotic complications and correction of raised intracranial pressure. Hyperuricemia can be managed by using allopurinol. Cytoreductive agent hydroxyurea or phosphodiesterase inhibitor anagrelide selective platelet-lowering agent protective against venous thrombosis may be used. Complete remission in polycythemia patient can be achieved by pegylated interferon-alpha. PV may be cured by allogeneic bone marrow transplantation. Phlebotomy alone could be effective for management of PV without functional impairment. Thorough clinical, hematological, biochemical, and radiological workup and prompt treatment should be initiated in any case presenting with CVST to prevent neurological complication and save life.

  Conclusion Top

CVST may be an initial presentation of PV. Hypercoagulable state caused by various disorders of formed blood elements such as PV, essential thrombocytosis, and sickle cell disease may lead to thrombosis of venous sinus. Hence, in young patients presenting with CVT, screening for prothrombotic entities is recommended.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Saroja AO, Tapsi C, Naik KR. Cerebral venous thrombosis in women from Indian subcontinent. J Sci Soc 2017;44:20-5.  Back to cited text no. 1
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Bousser MG. Cerebral venous thrombosis: Diagnosis and management. J Neurol 2000;247:252-8.  Back to cited text no. 4
Newton LK. Neurologic complications of polycythemia and their impact on therapy. Oncology (Williston Park) 1990;4:59-64.  Back to cited text no. 5
Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol 2005;128:275-90.  Back to cited text no. 6
Nagalla S, Besa EC, Talavera F: Polycythemia vera. Emedicine 2019. Available from: http://emedicine.medscape.com/article/205114-overview. [Last accessed on 2020 Jul 10].  Back to cited text no. 7
Spivak JL. Polycythemia vera: Myths, mechanisms, and management. Blood 2002;100:4272-90.  Back to cited text no. 8
Bagot CN, Arya R. Virchow and his triad: A question of attribution. Br J Haematol 2008;143:180-90.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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