Voulgarelis M, Giannouli S, Tasidou A, Anagnostou D, Ziakas P D, Tzioufas A G
University of Athens Medical School, Department of Pathophysiology, Athens, Greece.
Am J Hematol. 2006 Aug;81(8):590-7. doi: 10.1002/ajh.20593.
The histopathologic features characterizing the involvement of the bone marrow (BM) in systemic lupus erythematosus (SLE) have not been systematically analyzed to date.
The aim of this study was to assess morphologic and immunohistochemical characteristics of BM involvement in SLE.
Clinical and serological data of 40 SLE patients with unexplained cytopenias were studied. Ten patients with myelodysplasia of refractory anemia (RA) were used as controls. BM aspiration, BM biopsy (BMB), and immunohistochemistry were carried out in patients and controls. BM fibrosis, BM necrosis, stromal edema, and abnormal localization of immature precursors (ALIP) were assessed according to standard criteria.
Dyserythropoiesis and megakaryocytic atypias were uniform findings in SLE patients. The disruption of the normal BM architecture was a predominant SLE BM feature affecting cells of all three hemopoietic lineages, with both erythroid and megakaryocytic precursors tending to assume paratrabecular locations and ALIP aggregates being present in 27 cases. In addition, BM was hypocellular in 23 cases. BM necrotic alterations were evident in 90% of the cases. The density of reticulin content was generally increased. Vascular changes including dilatation of sinuses were manifest and were associated with the presence of necrotic alterations (P = 0.008). Hemoglobin levels correlated inversely with the presence of ALIP (P = 0.016). Upon comparing BMB features between SLE and RA controls there were striking similarities.
BMB in patients with SLE and unexplained cytopenias presents a variety of histopathologic findings including BM necrosis, stromal alterations, hypocellularity, dyspoiesis, and distortion of normal BM architecture, characterized primarily by the presence of ALIP aggregates.
迄今为止，尚未对系统性红斑狼疮（SLE）累及骨髓（BM）的组织病理学特征进行系统分析。
本研究旨在评估SLE累及BM的形态学和免疫组化特征。
研究了40例原因不明血细胞减少的SLE患者的临床和血清学数据。将10例难治性贫血（RA）骨髓发育异常患者作为对照。对患者和对照进行骨髓穿刺、骨髓活检（BMB）和免疫组化。根据标准标准评估骨髓纤维化、骨髓坏死、间质水肿和未成熟前体细胞异常定位（ALIP）。
红细胞生成异常和巨核细胞异型性是SLE患者的常见表现。正常BM结构破坏是SLE患者BM的主要特征，影响所有三个造血谱系的细胞，红系和巨核系前体细胞均倾向于位于小梁旁，27例存在ALIP聚集。此外，23例患者骨髓细胞减少。90%的病例有明显的骨髓坏死改变。网硬蛋白含量密度普遍增加。包括窦扩张在内的血管变化明显，且与坏死改变的存在相关（P = 0.008）。血红蛋白水平与ALIP的存在呈负相关（P = 0.016）。比较SLE患者与RA对照的BMB特征时，有显著相似之处。
SLE患者伴原因不明血细胞减少时，BMB呈现多种组织病理学表现，包括骨髓坏死、间质改变、细胞减少、造血异常和正常BM结构扭曲，主要特征为存在ALIP聚集。
