Zaninoni Anna, Imperiali Francesca G, Pomati Mauro, Colombi Mariangela, Boschetti Carla, Barcellini Wilma
U.O. Ematologia 2, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Clin Lab. 2010;56(9-10):459-62.
In this article we report a case of erythroblastic synartesis, a rare disease characterized by ineffective erythropoiesis, clusters of erythroblasts due to membrane invaginations, in which an autoimmune pathogenesis is hypothesized. We investigated the presence of anti-erythroblast autoimmunity in bone marrow cultures using a mitogen-stimulated direct antiglobulin test, a method reported to be able to disclose a latent autoimmunity in various diseases. The test revealed the presence of erythroblast-bound IgG, supporting the hypothesis of the autoimmune pathogenesis of erythroblastic synartesis. Supernatants induced the same specific morphological features, i.e erythroblastic clustering and diserythropoietic signs (multiple nuclei, nuclear inclusions, and intercellular bridges) in normal progenitors.
在本文中，我们报告了一例成红细胞联合症病例，这是一种罕见疾病，其特征为无效红细胞生成、因细胞膜内陷形成的成红细胞簇，推测其发病机制为自身免疫性。我们使用丝裂原刺激直接抗球蛋白试验研究骨髓培养物中抗成红细胞自身免疫的存在，该方法据报道能够揭示多种疾病中的潜在自身免疫。试验显示存在与成红细胞结合的IgG，支持成红细胞联合症自身免疫发病机制的假说。上清液在正常祖细胞中诱导出相同的特异性形态学特征，即成红细胞聚集和红细胞生成障碍体征（多核、核内包涵体和细胞间桥）。
