PDF(Pubmed)
Abstract：
This report of two cases confronts the longstanding perception of Sickle Cell Trait (SCT) as a clinically benign condition, highlighting its complex and severe clinical manifestations, particularly in the context of blood loss anemia and vaso-occlusive crises (VOCs). The hallmark of sickle cell disease is the severe pain caused by acute vaso-occlusion of the microvasculature that leads to bone marrow infarction. We report two cases of patients with SCT and severe anemia in the setting of blood loss secondary to uterine fibroids subsequently causing VOCs with likely bone sequestration. The occurrence of VOCs in SCT, while infrequent, can be serious and demands a high index of suspicion, particularly when patients appear in significant distress and cardiac or vascular etiologies are ruled out as a source. Reversal of anemia in this case provided quick resolution to symptoms, and we recommend other clinicians not disregard a differential of VOC in SCT carriers, and urge to treat patients as they would if they had sickle cell disease. This report challenges the conventional view of SCT as a condition of clinical benignity, calling for a recalibration in the clinical understanding, management strategies, and focus on this genetic trait under similar circumstances.
摘要：
这两例病例的报告长期认为镰状细胞特征（SCT）是临床上的良性疾病，突出了其复杂而严重的临床表现，特别是在失血性贫血和血管闭塞危象（VOCs）的情况下。镰状细胞疾病的标志是由微血管系统的急性血管闭塞引起的严重疼痛，导致骨髓梗塞。我们报告了两例SCT和严重贫血的患者，这些患者继发于子宫肌瘤的失血，随后导致VOC并可能发生骨隔离。SCT中VOCs的发生，虽然不常见，可能很严重，需要很高的怀疑指数，特别是当患者出现严重的痛苦和排除心脏或血管病因时。在这种情况下，贫血的逆转提供了快速解决的症状，我们建议其他临床医生不要忽视SCT携带者中VOC的差异，并敦促治疗患者，就像他们患有镰状细胞疾病一样。本报告挑战SCT作为临床良性条件的传统观点，呼吁重新校准临床理解，管理策略，并在类似情况下关注这种遗传性状。
