Schmalzing M, Aringer M, Bornhäuser M, Atta J
Medizinische Klinik & Poliklinik II, Universitätsklinikum Würzburg, Würzburg, Deutschland.
Medizinische Klinik III, Universitätsklinikum Carl Gustav Carus, Dresden, Deutschland.
Z Rheumatol. 2017 Oct;76(Suppl 2):26-32. doi: 10.1007/s00393-017-0369-2.
Myelodysplastic syndromes (MDS) represent a heterogeneous group of clonal hematopoietic stem cell disorders. They are characterized by inefficient hematopoiesis leading to peripheral cytopenia of one or more lineages and a variable risk of transformation into acute myeloid leukemia. They may either arise de novo as well as following exposition to environmental toxins, previous radiotherapy or chemotherapy or in the context of autoinflammatory diseases and related therapy. Characteristic cytogenetic abnormalities, along with the numbers of hematopoietic lineages affected and bone marrow blasts, enable an assessment of the risk of leukemic transformation. Acute leukemias are characterized by an accumulation of immature myeloid or lymphatic progenitor cells with limited differentiation capacity in the bone marrow. Proliferation of blast cells leads to suppression of normal hematopoiesis resulting in peripheral pancytopenia or leukocytosis associated with anemia and thrombocytopenia. Acute leukemias following MDS are defined as high-risk diseases. Intensive induction therapy followed by allogeneic stem cell transplantation is currently regarded as the only potentially curative treatment strategy. In this article the basic aspects of current diagnostics and treatment strategies for MDS and acute leukemia are outlined. Because of similarities with rheumatic inflammatory diseases, manifestations and treatment of graft versus host disease (GvHD) are also included.
骨髓增生异常综合征(MDS)是一组异质性的克隆性造血干细胞疾病。其特征为造血功能低下,导致一个或多个谱系的外周血细胞减少,以及转化为急性髓系白血病的风险各异。它们既可能原发出现,也可能在接触环境毒素、既往放疗或化疗后出现,或者在自身炎症性疾病及相关治疗的背景下发生。特征性的细胞遗传学异常,以及受影响的造血谱系数量和骨髓原始细胞数量,有助于评估白血病转化的风险。急性白血病的特征是骨髓中未成熟的髓系或淋巴祖细胞积聚,分化能力有限。原始细胞的增殖导致正常造血受到抑制,从而导致外周全血细胞减少或白细胞增多并伴有贫血和血小板减少。MDS后的急性白血病被定义为高危疾病。目前,强化诱导治疗后进行异基因干细胞移植被视为唯一可能治愈的治疗策略。本文概述了MDS和急性白血病当前诊断和治疗策略的基本方面。由于与风湿性炎症性疾病存在相似之处,还包括移植物抗宿主病(GvHD)的表现和治疗。