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Sickle Cell Disease: Considerations for the Cerebrovascular Neurosurgeon

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Management of Cerebrovascular Disorders

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Abstract

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy first described in 1915 by James Herrick characterized by anemia, vaso-occlusive crises, autosplenectomy, and susceptibility to infection. It is a result of a single transversion mutation (adenosine to thymine), wherein glutamic acid is replaced by valine at the sixth position in the beta globin subunit of hemoglobin (HbS) (Bunn, and Aster. Pathophysiology of blood disorders. McGraw-Hill Medical, New York, 2011). Non-affected hemoglobin is soluble within the intracellular milieu, allowing red blood cells to maintain their flexible structural integrity. Affected hemoglobin, however, will polymerize into an insoluble precipitate when exposed to certain stressors such as hypoxia or dehydration, resulting in a process of red blood cell morphologic change commonly referred to as “sickling.” Sickling can occur when erythrocytes pass through the capillaries, where oxygen saturation decreases, resulting in the alteration of the hemoglobin quaternary conformation, yielding the characteristic crescent morphology of affected RBCs (Bunn. N Engl J Med 337:762–769, 1997). In addition to this rigid structure of erythrocytes, studies indicate that sickled RBCs exhibit an affinity to vascular endothelium which promotes clot formation, ultimately ensuing vaso-occlusive crises (Kaul et al. J Clin Invest 96(6):2845–2853, 1995).

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Authors and Affiliations

  1. Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, USA

    Stephen R. Lowe, Mohammed Alshareef & Alejandro M. Spiotta

  2. Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA

    Julie Kanter

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  2. Mohammed Alshareef
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Correspondence to Alejandro M. Spiotta .

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Editors and Affiliations

  1. Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, USA

    Alejandro M. Spiotta

  2. Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, USA

    Raymond D. Turner

  3. Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, USA

    M. Imran Chaudry

  4. Department of Neurosurgery, Medical University of South Carolina, Charleston, SC, USA

    Aquilla S. Turk

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Lowe, S.R., Alshareef, M., Kanter, J., Spiotta, A.M. (2019). Sickle Cell Disease: Considerations for the Cerebrovascular Neurosurgeon. In: Spiotta, A., Turner, R., Chaudry, M., Turk, A. (eds) Management of Cerebrovascular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-99016-3_43

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