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| LETTER TO EDITOR |
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| Year : 2022 | Volume
: 70
| Issue : 4 | Page : 1717-1719 |
Polycythemia Vera Presenting as Subdural Hematoma
Indrajit Rana, Laxmi N Tripathy, Sunandan Basu
Department of Neurosurgery, MedicaSuperspecialty Hospital, Kolkata, West Bengal, India
| Date of Submission | 29-May-2019 |
| Date of Decision | 20-Jun-2019 |
| Date of Acceptance | 20-Mar-2020 |
| Date of Web Publication | 30-Aug-2022 |
Correspondence Address:
Indrajit Rana
Medica Superspecialty Hospital, 127 Eastern Metropolitan Bypass, Mukundapur, Kolkata, West Bengal- 700 099
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.355135
How to cite this article:
Rana I, Tripathy LN, Basu S. Polycythemia Vera Presenting as Subdural Hematoma. Neurol India 2022;70:1717-9 |
Sir,
Polycythemia vera (PCV) is a bone marrow disorder with excessive production of red blood cells. Patients with PCV are prone to the development of intravascular thrombosis. Major thrombotic complications (e.g. heart attack, stroke, deep venous thrombosis, or Budd-Chiari syndrome) may sometimes be the first symptom or indication that a person has PCV. However, spontaneous central nervous system (CNS) hemorrhage in PCV is rarely reported in the literature. Herein, we report a case of spontaneous subdural hematoma (SDH) in a previously undiagnosed PCV patient.
A 40 years old woman was presented with sudden onset headache associated with nausea and repeated episodes of vomiting. There was no history of trauma. At admission, her Glasgow Coma Scale (GCS) was 15/15, the pupil was equal bilaterally (2 mm) and briskly reacting to light. She had no gross motor and sensory deficit, no organomegaly or lymphadenopathy. She was not on regular oral contraceptives recently and conceived her last child 6 years back. Her blood pressure was 180/100 mm Hg, pulse rate 74/min, blood sugar 134 mg/dL, afebrile. A plain CT scan of the brain showed acute right-hemispheric SDH [Figure 1]. MR venography showed right transverse sinus thrombosis with prominent venous collaterals on the right side [Figure 2]. Her hemogram [Table 1] showed raised hemoglobin 17.1 gm/dL (normal 12.5 to 16.0) and RBC count 5.91 million/μL (normal 4.20 to 5.40). Her serum erythropoietin level was low i.e., 3.27 mIU/mL (Normal 4.30 to 29.00), which confirmed the diagnosis of PCV. She underwent phlebotomy and initially had conservative treatment for SDH. Subsequently, she developed an intense headache and left-sided weakness (4/5). Repeat CT scan showed an increase in SDH and mass effect [Figure 3]. She underwent right-sided mini craniectomy and drainage of SDH [Figure 4]. The post-operative period was uneventful and she had complete relief of headache. On genetic analysis, she was found to have V617F JAK 2 mutation. The bcr-abl translocation gene was negative. | Figure 2: MR Venogram showing right transverse sinus thrombosis with prominent venous collaterals in right side
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 | Figure 3: NCCT brain showing increase in size of right sided subdural haematoma and mass effect
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PCV causes a marked increase in the production of erythrocytes resulting in hyper-viscosity of blood which can cause various thromboembolic events. The frequency of venous events is less than for arterial ones. Thrombosis is more common (12 to 39%) than hemorrhagic complications (1.7 to 20%).[1]
Spontaneous SDH in PCV patients is rare.[2] Although the exact mechanism is not known, underlying micro-vascular thrombotic complications can result in spontaneous SDH. In our case, right transverse sinus thrombosis might be the cause of spontaneous right-sided SDH. Hemodynamic stress caused by cerebral venous sinus thrombosis can result in the collapse of a bridging vein. An acute subdural hematoma can result from cerebral venous bleeding due to obstructed venous return.[3] Sirin, et al.[1] reported concomitant cranial and spinal SDH as the initial presentation in the case of PCV. In a literature review, Akins, et al.[4] found many patients with SDH complicating cerebral venous thrombosis (CVT) have underlying thrombophilias. A higher venous thrombosis risk has been reported in patients with myeloproliferative disorders and the V617F JAK 2 mutation[5] and this mutation was present in our patient as well. When thrombophilia is diagnosed hematology consultation should be done to coordinate timing and duration for anticoagulation. CVT may arise from various other clinical factors and about one-third of patients with CVT do not have thrombophilia or contributing condition which is identified.[6]
To conclude, in the non-traumatic brain hemorrhage case, recognition of PCV as a possible cause of CVT should be kept in mind.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient and her parents have given consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| » References | |  |
| 1. | Sirin NG, Yesilot N, Ekizoglu E, Keles N, Tuncay R, Coban O, et al. A Case Report of Cerebral Venous Thrombosis in Polycythemia Vera Presenting with Intracranial and Spinal Subdural Hematoma. Case Rep Neurol 2010;2:37-45.  |
| 2. | Entezami P, Sanders LN, Raval MP, Adepoju A, Yamamoto J. Spontaneous Subdural Hematoma in a Patient with Polycythemia Vera. World Neurosurg 2019;125:354-6. |
| 3. | Takahashi S, Shinoda J, Hayashi T. Cerebral venous sinus thrombosis in an adult patient presenting as headache and acute subdural hematoma. J Stroke Cerebrovasc Dis 2012;21:338-40. |
| 4. | Akins PT, Axelrod YK, Ji C, Ciporen JN, Arshad ST, Hawk MW, et al. Cerebral venous sinus thrombosis complicated by subdural hematomas: Case series and literature review. Surg Neurol Int 2013;4:85-6. [ PUBMED] [Full text] |
| 5. | Malak S, Labopin M, Saint-Martin C, Bellanne-Chantelot C, Najman A, Gorin NC, et al. Long term follow up of 93 families with myeloproliferative neoplasms: Life expectancy and implications of JAK2V617F in the occurrence of complications. Blood Cells Mol Dis 2012;49:170-6. |
| 6. | Saposnik G, Barinagarrementeria F, Brown RD Jr, Bushnell CD, Cucchiara B, Cushman M, et al. American Heart Association Stroke Council and the Council on Epidemiology and Prevention.Diagnosis and management of cerebral venous thrombosis: A statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011;42:1158-92. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1]
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