Cerebral sinus thrombosis as an initial symptom of acute promyelocytic leukemia: Case report and literature review
- Department of Neurosurgery, Mount Sinai Hospital, New York City, United States.
- Department of Neurosurgery Albany Medical Center, Albany, New York, United States.
- Department of Neurology, Albany Medical Center, Albany, New York, United States.
- Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, United States.
Laura Salgado-Lopez, Department of Neurosurgery, Mount Sinai Hospital, New York City, New York, United States.
DOI:10.25259/SNI_958_2021Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Laura Salgado-Lopez1, Amanda Custozzo2, Nataly Raviv2, Tamer Abdelhak3, Maria Peris-Celda4. Cerebral sinus thrombosis as an initial symptom of acute promyelocytic leukemia: Case report and literature review. 11-Mar-2022;13:89
How to cite this URL: Laura Salgado-Lopez1, Amanda Custozzo2, Nataly Raviv2, Tamer Abdelhak3, Maria Peris-Celda4. Cerebral sinus thrombosis as an initial symptom of acute promyelocytic leukemia: Case report and literature review. 11-Mar-2022;13:89. Available from: https://surgicalneurologyint.com/surgicalint-articles/11433/
Background: Cerebral sinus thrombosis as presentation of acute promyelocytic leukemia (AMPL) is exceptional, with only three cases registered in the literature.
Case Description: A 24-year-old female patient was transferred to our center after a car accident. The patient had a witnessed generalized seizure while driving. Computerized tomography (CT) demonstrated a temporal intraparenchymal hemorrhage and CT venogram diagnosed a cerebral sinus thrombosis on the left transverse and sigmoid sinus. The patient underwent surgical evacuation of the hematoma and was treated with anticoagulation 48 h after surgery. Pancytopenia alerted of a possible hematological disorder. The patient was subsequently diagnosed with AMPL and treated with arsenic trioxide. The patient had a complete neurological recovery with no postoperative complications.
Conclusion: The management of cerebral sinus thrombosis in patients with AMPL remains controversial. The previous reported cases of cerebral sinus thrombosis preceding the diagnosis of AMPL are reviewed and treatment of cerebral sinus thrombosis with anticoagulation in the setting of intraparenchymal hemorrhage and bleeding disorders is also discussed.
Keywords: Acute promyelocytic leukemia, Cerebral sinus thrombosis, Intraparenchymal hemorrhage, Seizure
Cerebral sinus venous thrombosis (CVT) is a rare but potentially devastating type of stroke, accounting for approximately 0.5% of them.[
While bleeding is a characteristic feature of APML, reports of CVT in the setting of APML remain exceptional. Within them, CVT usually occurs during the induction treatment with all-trans retinoic acid, or later in the course of the disease.[
A 24-year-old female was admitted to the emergency room after a motor vehicle accident caused by a witnessed generalized seizure while driving. An initial computerized tomography (CT) scan of the head showed a left temporal intraparenchymal hemorrhage [
Case illustration. (a) Preoperative CT scan of the head performed on arrival to our center demonstrating a left intraparenchymal hemorrhage of 47 × 31 × 60 mm, with mass effect on adjacent brain structures and a midline shift of 6 mm. (b) Preoperative cranial CT angiography, venous phase, showing right transverse and sigmoid sinuses patency and occlusion of the left transverse (arrowhead) and sigmoid (arrow) sinuses. (c) Preoperative cranial CT angiography, venous phase, showing right sigmoid sinus and jugular vein patency and occlusion of the sigmoid sinus (arrow) and jugular vein (arrowhead) on the left side. (d) Postoperative CT scan performed 24 h after the surgical procedure showing resolution of the midline shift and evacuation of the temporal hemorrhage through a left craniotomy with minimal amount of pneumocephalus.
The patient’s initial blood test revealed pancytopenia that worsened over the 1st days: the initial white blood cell count was 2 × 10/l, with a red blood cell count of 3.43 × 10/l, platelets of 13 × 10/l, and a hemoglobin count at 10.6 g/dl; blood test results 2 days after admission showed a white blood cell count of 1.7 × 10/l, a red blood cell count of 2.14 × 10/l, platelets of 10 × 10/l, and a hemoglobin count at 6.7 g/dl, therefore, the patient received one unit of red blood cells. A peripheral smear displayed features suggestive of APML. Initial testing for Factor V Liden, prothrombin gene mutation, hepatitis C panel, HIV, antinuclear Ab, anticardiolipin IgG/IgM/IgA, beta-2glycoprotein G/M/ IgA, MTHFR, and JAK2 V617F was all negative. A bone marrow biopsy was performed on the 4th postoperative day.
The fluorescence in situ hybridization analysis of the bone marrow sample revealed dual fusion signals in the 15;17 probe set, indicating PML/RARA fusion and confirming the diagnosis of APML. The treatment with arsenic trioxide was consequently started, and the heparin drip was switched to subcutaneous low-molecular-weight heparin (LMWH) at a therapeutic dose 2 weeks after the surgery. She experienced a progressive neurological recovery with complete resolution of the motor dysphasia and right upper limb paresis, and is currently completing the antineoplastic treatment with good response.
CVT is an uncommon and frequently unrecognized type of stroke. Clinical findings are usually either related to increased intracranial pressure due to impaired venous drainage or to focal brain injury from venous ischemia or hemorrhage.[
Predisposing factors of CVT are multiple and can be identified in up to 80% of patients.[
APML is a distinct subtype of acute myeloid leukemia, with symptoms usually related to the characteristic underlying pancytopenia, such as fatigue, infections, and bleeding. Hence, intracranial events are usually hemorrhagic rather than thrombotic.[
MR imaging in combination with MR venography has largely replaced invasive cerebral angiography in the diagnosis of CVT and is usually considered the technique of choice for diagnostic evaluation and follow-up. However, CT scan imaging is preferred over MR to assess patients with suspected intracranial hemorrhage and cerebral CT venography has proven to be equivalent to MR venography in the diagnosis of CVT.[
The treatment of CVT still lacks an uniform approach.[
The management of a stroke or major thrombosis in APML remains challenging, given that intracerebral and pulmonary hemorrhages are the most frequent causes of death due to the complex coagulopathy associated with APML.[
Management of cerebral sinus thrombosis in patients with acute promyelocytic leukemia remains controversial. Previous reported cases of cerebral sinus thrombosis preceding the diagnosis of acute promyelocytic leukemia are reviewed, and treatment of cerebral sinus thrombosis with anticoagulation in the setting of intraparenchymal hemorrhage and bleeding disorders are also discussed.
All aspects of this study were approved by the Institutional Review Board of the Albany Medical Center, Albany, NY.
Patient’s consent not required as patients identity is not disclosed or compromised.
There are no conflicts of interest.
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