Urbanowicz Iwona, Nahaczewska Wiesława, Celuch Beata
Department of Clinical Chemistry, Department of Hematology, Wroclaw Medical University, Wrocław, Poland. Email:
Department of Clinical Chemistry, Department of Hematology, Wroclaw Medical University, Wrocław, Poland.
Ann Palliat Med. 2021 Jan;10(1):694-699. doi: 10.21037/apm-20-1957. Epub 2020 Dec 17.
Aplastic anemia (AA) is a rare, life-threatening syndrome of bone marrow failure resulted from bone marrow hypoplasia or aplasia, leading to pancytopenia (not only anemia). The most common cause is an autoimmune reaction of T lymphocytes against hematopoietic stem cells or, less frequently, a congenital defect or acquired damage to these cells, which leads to inhibition of their division and differentiation. AA can develop quickly (within a few days) or slowly (several weeks or months). The signs and symptoms are related to anemia, neutropenia, and thrombocytopenia. The concepts of treatment of patients with AA have significantly evolved in recent years. This is due to improved outcomes of both family and unrelated donor hematopoietic stem cell transplantations (HSCTs) as well as to revised results of immunosuppressive therapy (IST). The choice of the method depends essentially on three factors: the severity of AA, the age of the patient, matched sibling donor. All patients diagnosed with AA require appropriate supportive treatment adapted to the current clinical situation. Supportive treatment is necessary both before, during and after invasive causal treatment, it mainly involves the transfusion of leukocyte-depleted blood components, the use of anti-infectious prophylaxis or treatment of infections. In many cases AA, supportive therapy is the only therapeutic option, especially in elderly patients with comorbidities. In this paper we present current supportive treatment in this life-threatening disease.
再生障碍性贫血（AA）是一种罕见的、危及生命的骨髓衰竭综合征，由骨髓发育不全或发育不良引起，导致全血细胞减少（不仅是贫血）。最常见的原因是T淋巴细胞对造血干细胞的自身免疫反应，或较少见的这些细胞的先天性缺陷或获得性损伤，这会导致其分裂和分化受到抑制。AA可快速发展（在几天内）或缓慢发展（几周或几个月）。其体征和症状与贫血、中性粒细胞减少和血小板减少有关。近年来，AA患者的治疗观念有了显著发展。这是由于亲缘和非亲缘供者造血干细胞移植（HSCT）的疗效提高，以及免疫抑制治疗（IST）结果的修正。治疗方法的选择主要取决于三个因素：AA的严重程度、患者年龄、匹配的同胞供者。所有诊断为AA的患者都需要根据当前临床情况进行适当的支持治疗。在进行侵入性病因治疗之前、期间和之后，支持治疗都是必要的，主要包括输注去除白细胞的血液成分、使用抗感染预防措施或治疗感染。在许多AA病例中，支持治疗是唯一的治疗选择，尤其是在患有合并症的老年患者中。在本文中，我们介绍了这种危及生命疾病的当前支持治疗方法。
