Madam A, a 72-year-old lady, was diagnosed with JAK2 V617F mutation polycythemia vera in 2019 and was treated with tablet hydroxyurea 500 mg daily and oral aspirin 75 mg daily. She required therapeutic phlebotomy once a week for the first month as her hematocrit level was persistently more than 50%. Prior to her diagnosis of polycythemia vera, she had a history of ischemic stroke in 2008 with residual left-sided hemiparesis, hemorrhoids with banding done twice, diabetes mellitus, hypertension, and dyslipidemia.
In early March 2020, during the COVID-19 outbreak in Malaysia, most of her family members whom she was staying with were infected with COVID-19. Therefore, she was screened for being a close contact and was tested positive for SARS-CoV-2 by qualitative real-time reverse-transcriptase-polymerase-chain-reaction (qRT-PCR) assay. She was admitted to the isolation ward for close observation as per local COVID-19 protocol. Her treatment of aspirin and hydroxyurea for her underlying polycythemia vera was continued. While under observation, she developed a worsening cough with chest radiograph showed worsening bilateral lower zone opacities. However, her oxygen saturation remained stable at 97 to 100% under room air. Due to the severity of her COVID-19 infection, she was commenced on oral hydroxychloroquine for 5 days and oral lopinavir/ritonavir for 2 weeks. Her clinical condition improved with treatment. However, she developed per rectal bleeding which was attributed to her hemorrhoids. Her hemoglobin was 16.6 g/dL, total white cell count was 25 x 103/mul, and platelet count was 1247 x 103/mul at the time the bleeding occurred (Table 1). As a result, her hydroxyurea dosage was increased to 500 mg twice daily but her aspirin was withheld. Her rectal bleeding resolved after that. She was hospitalized for 40 days and was discharged following a negative COVID-19 swab. She was not commenced on any anticoagulant in view of the history of rectal bleeding.
One week post discharge, she was readmitted with sudden onset of altered consciousness associated with right-sided body weakness. Her Glasgow Coma Scale (GCS) was 14/15, E3V5M6 on presentation. Computed tomography (CT) brain was performed and revealed cerebral venous thrombosis of the straight sinus, vein of Galen, and bilateral internal cerebral veins with venous infarcts (Fig. 1). She was commenced on subcutaneous (S/C) enoxaparin 40 mg twice a day (weight 42 kg). On day 3 of admission, she had a repeat CT brain and cerebral venography for fluctuating GCS level. The repeat brain scan showed evidence of thrombosis involving the superior sagittal and right sigmoid sinus as well, bilateral vasogenic edema, swollen left basal ganglia, and left thalamic region. She was started on intravenous dexamethasone for 5 days to reduce cerebral edema and oral levetiracetam for seizure prophylaxis.
Her GCS started to improve 2 days after the commencement of the above treatment. She had a repeat non-contrasted CT brain a week after her 2nd CT brain and showed unchanged multifocal infarcts, no intracranial hemorrhage, and less swollen left basal ganglia region. At the time of her discharge, her GCS was 15/15 and she was on nasogastric tube feeding and wheel-chaired bound due to incomplete recovery of her motor function. On her clinic follow-up a month later, her D-dimer was normal at 0.24mug/ml, and her S/C enoxaparin 40 mg BD was converted to oral warfarin. She is clinically stable on her last review 3 months later.