Umar Zaryab, Ilyas Usman, Nso Nso
Internal Medicine, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, New York, USA.
Department of Medicine, Ichan School of Medicine at Mount Sinai, NYC Health and Hospitals, New York, USA.
Cureus. 2022 Mar 29;14(3):e23604. doi: 10.7759/cureus.23604. eCollection 2022 Mar.
Sickle cell disease is an autosomal recessive disorder resulting in the substitution of CTG by CAG in the sixth codon of the beta-globin gene. As a result of this, the hydrophilic glutamic acid residue is replaced by hydrophobic valine residue, leading to the formation of hemoglobin tetramer HBS. This alteration in the beta-globin chain makes the red blood cells prone to sickling, especially in the presence of risk factors such as stress, hypoxia, and infection. These sickled red blood cells have the tendency to adhere to the endothelium and lead to vessel occlusion and distal tissue ischemia. The recent coronavirus disease 2019 (COVID-19) outbreak has impacted millions across the globe, putting individuals with co-morbidities at particularly high risk, and patients with sickle cell disease are no exception. We present the case of a 47-year-old African American male presenting to the emergency department with subjective fevers and a two-day history of pain in the arms, legs, and chest. A diagnosed case of sickle cell disease, the patient was on hydromorphone for pain management but ran out of his medications a few weeks before presentation. On examination, the patient was saturating well with mild tenderness upon palpation of the arms, legs, and chest. On complete blood count, the patient had a hemoglobin of 11.3 g/dL and a white cell count of 13.1 x10(3)/mcL. The patient had a normal mean corpuscular volume with reticulocytosis, hypochromia, ovalocytosis, poikilocytosis, polychromasia, and target cells. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) polymerase chain reaction (PCR) was positive. The chest X-ray did not reveal any significant findings. He was admitted to the medicine floor for the management of sickle cell crisis and was placed under airborne and droplet precautions. The patient was started on hydromorphone for pain management and intravenous fluid hydration. On the second day of admission, the patient reported increasing shortness of breath. He was saturating 90% on room air and 94% on 2 liters of supplemental oxygen. The white blood cell count increased to 18.42 x10(3)/mcL and the chest X-ray revealed reticular densities with patchy alveolar opacities in the left lung. Given the decline in respiratory status, the patient was started on remdesivir. Over the course of his hospital stay, the patient's pain and respiratory status improved, with the patient saturating 97% on room air. He was discharged home with instructions to follow isolation precautions for at least two weeks, folic acid, and adequate pain management. An appointment was also scheduled for the patient to follow with a sickle cell nurse practitioner upon discharge.
镰状细胞病是一种常染色体隐性疾病，它导致β-珠蛋白基因第六密码子中的CTG被CAG取代。由此，亲水性的谷氨酸残基被疏水性的缬氨酸残基所替代，导致血红蛋白四聚体HBS的形成。β-珠蛋白链的这种改变使红细胞易于镰变，尤其是在存在压力、缺氧和感染等危险因素的情况下。这些镰变的红细胞易于黏附在内皮上，导致血管闭塞和远端组织缺血。最近的2019冠状病毒病（COVID-19）疫情影响了全球数百万人，使患有合并症的个体面临特别高的风险，镰状细胞病患者也不例外。我们报告一例47岁非裔美国男性病例，该患者因自觉发热以及手臂、腿部和胸部疼痛两天而就诊于急诊科。该患者被诊断为镰状细胞病，一直在使用氢吗啡酮进行疼痛管理，但在就诊前几周药物用完。检查时，患者血氧饱和度良好，手臂、腿部和胸部触诊时有轻度压痛。血常规检查显示，患者血红蛋白为11.3 g/dL，白细胞计数为13.1×10³/mcL。患者平均红细胞体积正常，但存在网织红细胞增多、低色素、椭圆形红细胞、异形红细胞、多染性红细胞和靶形细胞。严重急性呼吸综合征冠状病毒2（SARS-CoV-2）聚合酶链反应（PCR）呈阳性。胸部X线检查未发现任何明显异常。他因镰状细胞危象的管理被收入内科病房，并采取空气传播和飞沫隔离预防措施。患者开始使用氢吗啡酮进行疼痛管理并接受静脉补液。入院第二天，患者报告呼吸急促加重。他在室内空气中血氧饱和度为90%，在补充2升氧气时为94%。白细胞计数增至18.42×10³/mcL，胸部X线显示左肺有网状密度影伴斑片状肺泡实变。鉴于呼吸状况恶化，患者开始使用瑞德西韦治疗。在住院期间，患者的疼痛和呼吸状况有所改善，在室内空气中血氧饱和度达到97%。他出院回家，并被告知至少要遵循隔离预防措施两周，服用叶酸，并进行充分的疼痛管理。出院时还为患者安排了随访镰状细胞病专科护士从业者的预约。
