Shankar Karthik, Shah Deep, Huffman Deanna L, Peterson Chelsea, Bhagavatula Rama
Internal Medicine, Allegheny Health Network, Pittsburgh, USA.
Hematology/Oncology, Allegheny Health Network, Pittsburgh, USA.
Cureus. 2021 Jan 30;13(1):e13017. doi: 10.7759/cureus.13017.
Sickle cell anemia patients often present to the hospital with acute vaso-occlusive pain crisis. Symptoms can include, but are not limited to, chest pain, abdominal pain, and musculoskeletal pain. These symptoms are brought about due to the pathology of the disease. Abnormal hemoglobin S causes red blood cells to band together, otherwise known as "sickling." These patients also often present with very low hemoglobin levels on initial evaluation. In most cases, packed red blood cell transfusions are needed in order to replenish these patient's functional hemoglobin supply. Unfortunately, transfusing sickle cell patients can lead to an unwanted consequence, that of hyperhemolysis syndrome, in which blood transfusions prompt further hemolysis of the already sickled red blood cells. When this complication arises, caution must be exercised in deciding the next steps of treatment.
镰状细胞贫血患者常因急性血管阻塞性疼痛危象而入院。症状可能包括但不限于胸痛、腹痛和肌肉骨骼疼痛。这些症状是由该疾病的病理状况引起的。异常的血红蛋白S会导致红细胞聚集在一起,即所谓的“镰变”。这些患者在初次评估时血红蛋白水平通常也很低。在大多数情况下,需要输注浓缩红细胞以补充这些患者的功能性血红蛋白供应。不幸的是,给镰状细胞病患者输血可能会导致一种不良后果,即高溶血综合征,输血会促使已经镰变的红细胞进一步溶血。当出现这种并发症时,在决定下一步治疗措施时必须谨慎行事。