By: Dr. Sean Coulson
HPI: Pt is a 38 y/o male with hx of sickle cell (SS) who presents for generalized headache, facial droop and difficulty speaking. Last known normal was 19 hours PTA. No anticoagulation.
Objective: HR 91, BP 155/91, RR 20, Temp 98F, Accu Check 129 NIH 2 – mild left lower facial droop and mild dysarthria. No other neurologic deficits. Stroke alert is called and CT CTA do not show any acute hemorrhagic infarct or large vessel occlusion. Labs and EKG are sent and the only pertinent result includes a Hb of 6.1 (prior baseline 9).
DDX: acute hemorrhagic / ischemic stroke, seizure, dural venous sinus thrombosis, myasthenia gravis, botulism, complex migraine etc.
Management: Given that this patient was outside of the 4.5 hour window and there was a low suspicion for thromboembolic etiology, TNK was held. In discussion with neurology and hemonc, a shiley dialysis catheter was placed and exchange transfusion was initiated.
Exchange transfusion: Goal is to reduce HbS to < 30% preventing further sickling. In hemorrhagic stroke (primary or secondary to an ischemic stroke), exchange transfusion is still indicated however the goal is to prevent secondary vasospasm and recurrent strokes – data is less clear in terms of overall benefit. If exchange transfusion is not possible, simple regular transfusions are indicated immediately and can act as a bridge until exchange transfusion is established. Hb goal determined in discussion w/ Hematology.
Pearls:
~ Sickle cell is a common cause of acute stroke in children and young adults.
~ SS causes increased risk for cerebral aneurysms and other arterial abnormalities (high risk for carotid dissection, subarachnoid, dural venous thrombosis etc)
~ It is still important in acute strokes to apply general sickle cell crisis management (O2, fluids, pain control, transfusion)
~ Exchange transfusions lower risk of recurrent stroke
~ SS is not an absolute contraindication to TPA
*** Thrombolytics can be given in sickle cell patients if there is a high pretest probability for thromboembolic etiologies of the acute stroke (CAD, PVD, Afib etc) and the patient meets typical inclusion/exclusion criteria. However the benefit of thrombolytics in stroke secondary to sickled RBCs is questionable – data is limited.
~ If exchange transfusion is not possible, discussion w/ hematology and neurology about Hb parameters is paramount.
References
Hulbert ML, Scothorn DJ, Panepinto JA, et al. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. J Pediatr. 2006;149(5):710-712. doi:10.1016/j.jpeds.2006.06.037
Alakbarzade V, Maduakor C, Khan U, Khandanpour N, Rhodes E, Pereira AC. Cerebrovascular disease in sickle cell disease. Pract Neurol. 2023;23(2):131-138. doi:10.1136/pn-2022-003440
Tintinalli J. TINTINALLI’S EMERGENCY MEDICINE : A Comprehensive Study Guide. Mcgraw-Hill Education; 2019:1136.
EM:RAP CorePendium. EM:RAP CorePendium. Published 2024. Accessed August 10, 2024. https://www.emrap.org/corependium/chapter/recZWicqx0K20uwsz/Sickle-Cell-Disease#h.1h9pp1rguxde
