Găman Amelia, Găman G, Bold Adriana
Department of Pathophysiology, Faculty of Medicine, University of Medicine and Pharmacy of Craiova, Romania.
Rom J Morphol Embryol. 2009;50(4):669-74.
Aplastic anemia is a clonal disease of stem cell characterized by peripheral blood pancytopenia with hypocellular bone marrow. In most cases acquired aplastic anemia is an autoimmune, T-cell mediated disease (hematopoiesis is mediated by a population of CD8+ T-cells which produce inhibitory cytokines - TNF-alpha, IFN-gamma, IL-6 which suppress hematopoiesis by affecting the mitotic cycle and cell killing by inducing apoptosis). In some cases radiation, medical drugs and chemicals, viruses induce depletion of hematopoietic stem cells by direct toxicity; immune diseases induce complex immune reactions leading to bone marrow failure. Symptoms and signs are represented by fatigue, pallor induces by anemia, infections induce by neutropenia, and bleedings induce by thrombocytopenia. In peripheral blood is present pancytopenia and bone marrow are characterized by hypocellularity, fat cells hyperplasia, residual lymphocytosis, plasmocytosis and mastocytosis. The aim of this study was to establish the correlation between etiology, pathophysiology, bone marrow histology and negative prognosis factors at 16 patients with acquired aplastic anemia (seven with severe aplastic anemia and nine with moderate aplastic anemia) hospitalized in Clinic of Hematology from Craiova between 2003-2008. Eight cases presented idiopathic aplastic anemia and eight cases secondary aplastic anemia (two of them with pure red cell aplasia).
The unfavorable evolution, correlated with etiology and pathophysiology, had been seen at the patients with severe idiopathic aplastic anemia and severe secondary aplastic anemia associated with viral infections and insecticides exposure. Pure red cell aplasia was associated in our study with B19 parvovirus infection or malignant thymoma. The negative prognosis factors in acquired aplastic anemia, correlated with laboratory findings and a low survival, were: severe neutropenia, platelets count less than 10 000/microL, corrected reticulocytes less than 1%, hypocellularity of bone marrow <10%, persistence of pancytopenia at 30 days after initiating therapy.
再生障碍性贫血是一种干细胞克隆性疾病,其特征为外周血全血细胞减少伴骨髓细胞减少。在大多数情况下,获得性再生障碍性贫血是一种自身免疫性、T细胞介导的疾病(造血由一群产生抑制性细胞因子——肿瘤坏死因子-α、干扰素-γ、白细胞介素-6的CD8 + T细胞介导,这些细胞因子通过影响有丝分裂周期并诱导凋亡来抑制造血)。在某些情况下,辐射、药物、化学物质、病毒通过直接毒性导致造血干细胞耗竭;免疫性疾病引发复杂的免疫反应导致骨髓衰竭。症状和体征表现为疲劳、贫血引起的面色苍白、中性粒细胞减少引起的感染以及血小板减少引起的出血。外周血中存在全血细胞减少,骨髓的特征为细胞减少、脂肪细胞增生、残留淋巴细胞增多、浆细胞增多和肥大细胞增多。本研究的目的是确定2003年至2008年在克拉约瓦血液学诊所住院的16例获得性再生障碍性贫血患者(7例重度再生障碍性贫血和9例中度再生障碍性贫血)的病因、病理生理学、骨髓组织学与不良预后因素之间的相关性。8例为特发性再生障碍性贫血,8例为继发性再生障碍性贫血(其中2例为纯红细胞再生障碍)。
在重度特发性再生障碍性贫血以及与病毒感染和接触杀虫剂相关的重度继发性再生障碍性贫血患者中,观察到了与病因和病理生理学相关的不良病情发展。在我们的研究中,纯红细胞再生障碍与B19细小病毒感染或恶性胸腺瘤有关。获得性再生障碍性贫血的不良预后因素与实验室检查结果及低生存率相关,包括:重度中性粒细胞减少、血小板计数低于10000/微升、校正网织红细胞低于1%、骨髓细胞减少<10%、治疗开始后30天全血细胞减少持续存在。