Edwards April, Clay E Leila Jerome, Jewells Valerie, Adams Stacie, Crawford Regina D, Redding-Lallinger Rupa
Departments of Pediatrics and Internal Medicine, Division of Hematology and Oncology, University of North Carolina School of Medicine, 170 Manning Drive 1185A, Physician Office Building CB#7236, Chapel Hill, NC 27599-7236, USA.
J Med Case Rep. 2013 Aug 23;7:210. doi: 10.1186/1752-1947-7-210.
Vasculopathy of the large vessels commonly occurs in sickle cell disease, and as a result cerebral infarction is a well characterized complication of this condition. However, spinal infarction appears to be rare. Spinal infarct is infrequent in the non-sickle cell population as well, and accounts for only about 1 percent of all central nervous system infarcts.
In the present work, we report the case of a 19-year-old African-American man with sickle cell disease who experienced an anterior spinal infarct and subsequent quadriplegia. He was incidentally noted to be a heterozygote for factor V Leiden. We also reviewed the literature and found two previous cases of spinal cord infarction and sickle hemoglobin. Our literature search did not demonstrate that heterozygocity for factor V Leiden plays an important role in spinal cord infarction.
The paucity of cases associated with sickle hemoglobin does not allow us to postulate any particular risk factors with sickle cell disease that might predispose patients to spinal cord infarction. Our patient's case raises the question as to whether spinal cord infarction is being missed in individuals with sickle cell disease and neurologic symptoms.
大血管血管病变在镰状细胞病中较为常见,因此脑梗死是该疾病一种特征明确的并发症。然而,脊髓梗死似乎较为罕见。脊髓梗死在非镰状细胞人群中也不常见,仅占所有中枢神经系统梗死的约1%。
在本研究中,我们报告了一名19岁患有镰状细胞病的非裔美国男性,他发生了前脊髓梗死并随后出现四肢瘫痪。偶然发现他是凝血因子V莱顿突变杂合子。我们还查阅了文献,发现了另外两例脊髓梗死合并镰状血红蛋白的病例。我们的文献检索未显示凝血因子V莱顿突变杂合性在脊髓梗死中起重要作用。
与镰状血红蛋白相关的病例较少,这使我们无法推测镰状细胞病中可能使患者易患脊髓梗死的任何特定危险因素。我们患者的病例引发了一个问题,即患有镰状细胞病和神经系统症状的个体是否漏诊了脊髓梗死。