A 25 y.o. male with sickle cell disease presents with 3 weeks of HA
When he initially presented his head CT was read as normal. He then had the MRI shown below.
What does it show?
Our patient had a spontaneous epidural hematoma caused by his sickle cell disease.
A typical smear during a sickle cell crisis.
Evolutionarily, sickle cell disease provides a survival advantage against malaria fatality over people with normal hemoglobin and the mutation occurs in Africa predominantly in areas with Falciparum malaria.
Sickle cell disease is caused by a single amino acid substitution on the beta globin chain causing the hemoglobin molecule to polymerize in the deoxygenated state. Resultant hemolysis and microvascular injury can result. Neurologic manifestations affect 35% patients with 4% suffering ischemic strokes and 34% suffering intracranial bleeding. When other causes of bleeding are ruled out; aneursyms, infection, dural mets, coagulopathy from renal disease and Langerhans cell histiocytosis , sickle cell disease itself can be the cause.
Explanations for this spontaneous bleeding in sickle cell patients have been postulated as bony infarct with leakage of blood or sludging of sickle cells in the veins causing elevated back pressure and rupture of a thinned inner cortex.
“puff of smoke” shows on the arteriogram in Moyamoya disease where irregular perforating vascular networks form near occluded or stenotic vessel predispose to bleeding in sickle cell disease. Extracranial to intracranial bypass is often recommended.
While stroke and CNS disease remain the major complications of sickle cell disease, vaso-occlusive crisis is what is predominately seen in the adult ED. Other complications are related to chronic hemolysis, chronic end-organ damage, nitric oxide depletion and immune compromise from functional asplenia.
COMPLICATIONS OF SICKLE CELL DISEASE
Acute chest is a sickle crisis that produces an infiltrate on CXR. this can be due to infection or infarction.
Treatments
In 2021 a four year trial began taking stem cells from patients with sickle cell disease and using CRISPR to remove the defective gene and replace it with the correct sequence. The old marrow is then destroyed and the new repaired stem cells are infused. Unfortunately the trial was paused in jan 2023 because of prolonged low blood cell counts requiring ongoing transfusion and growth factor support. Other trials have reported the development of AML and malignancies. Currently, the only treatment approved for sickle cell disease is bone marrow transplant.
Additional approved agents include L-glutamine which forms NADH and is thought to relieve oxidant stress.and crizanlizumab which has been approved to prevent cellular adhesion and prevent painful crises. Hydroxurea increases fetal hemoglobin and has been shown to prolong life expectancy because it generate nitric oxide.
Our patient was thought to have a spontaneous bleed because he had no other explanation for bleeding and had a negative arteriogram. He underwent surgery and recovered uneventfully.
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Saha B, Saha A. Spontaneous epidural hemorrhage in sickle clell disease, are they all the same? A case report and comprehensive review of the literature. Case Rep Hematol. 2019;2019:8974580
Published Online: January 18, 2023. doi:10.1001/jama.2022.24496
Babatola B, Salman U , Abiola A, et al. Spontaneous epidural haematoma in sickle cell anaemia: case report and literature review. J Surg Tech Case Rep. 2012Jul-Dec;4(2):135-7.
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