PDF(Pubmed)
Abstract：
Warm autoimmune hemolytic anemia (WAIHA) is a rare disease. Roughly half of all cases are considered either primary or idiopathic. The remaining cases are typically secondary to a drug reaction or an underlying disease state such as malignancy, infection, or chronic autoimmune disease. Treatments for WAIHA include corticosteroids, intravenous immunoglobulin (IVIG), rituximab, and splenectomy. We present a case of WAIHA with underlying clear cell renal cell carcinoma (RCC) that was unresectable, creating a difficult treatment course. A 76-year-old male with recently diagnosed clear cell RCC was admitted with symptomatic WAIHA and significant hemodynamic instability. Over the course of his admission, he received 25 blood transfusions, erythropoietin, methylprednisolone, IVIG, rituximab, and mycophenolate mofetil in an attempt to control his disease state. He suffered end-organ damage in the form of heart failure with reduced ejection fraction. He was deemed too unstable for RCC resection or interventional cardiac procedures. Determining an appropriate transfusion threshold proved to be a noteworthy challenge. His hemoglobin eventually stabilized to 7.4 g/dL upon discharge over the course of 27 days of treatment. The underlying cause of his WAIHA was believed to be most likely secondary to RCC. WAIHA may have a prolonged treatment course with high risk of mortality if the underlying cause is not resolvable. If this is the case, it can be difficult to determine a hemoglobin transfusion threshold that maintains normal vital signs while minimizing the risk of transfusion-associated circulatory overload (TACO) and transfusion-related acute lung injury (TRALI). Prolonged hemodynamic instability may result in end-organ damage. For our patient, we aimed for a hemoglobin transfusion threshold of 5.0-6.0 g/dL based on his mean arterial pressure (MAP), heart rate, and subjective symptoms.
摘要：
温本身免疫性溶血性贫血(WAIHA)是一种罕见疾病。大约一半的病例被认为是原发性或特发性。其余病例通常继发于药物反应或潜在的疾病状态，如恶性肿瘤，感染,或慢性自身免疫性疾病。WAIHA的治疗包括皮质类固醇，静脉注射免疫球蛋白（IVIG），利妥昔单抗,和脾切除术.我们提出了一个WAIHA的病例，其潜在的透明细胞肾细胞癌（RCC）是不可切除的，创造了一个艰难的治疗过程。一名最近诊断为透明细胞RCC的76岁男性因有症状的WAIHA和显着的血流动力学不稳定而入院。在他承认的过程中，他接受了25次输血,促红细胞生成素,甲基强的松龙,IVIG,利妥昔单抗,和霉酚酸酯试图控制他的疾病状态。他以心力衰竭的形式遭受终末器官损伤，射血分数降低。对于RCC切除或心脏介入手术，他被认为太不稳定。确定适当的输血阈值被证明是一个值得注意的挑战。在27天的治疗过程中，他的血红蛋白最终稳定在7.4g/dL。他的WAIHA的根本原因被认为是RCC的次要原因。如果根本原因无法解决，WAIHA的治疗过程可能会延长，死亡风险很高。如果是这样的话,在维持正常生命体征的同时将输血相关循环超负荷(TACO)和输血相关急性肺损伤(TRALI)风险降至最低的血红蛋白输注阈值可能很困难.长时间的血流动力学不稳定可能导致终末器官损伤。对我们的病人来说,根据他的平均动脉压（MAP），我们的血红蛋白输血阈值为5.0-6.0g/dL，心率,主观症状。
