Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature
Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature
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DOI：
10.1016/j.humpath.2014.07.017
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发表时间：
2014-11-01
期刊：
影响因子：
3.3
通讯作者：
Brynes, Russell K.
中科院分区：
文献类型：
--
作者：
Vergara-Lluri, Maria E.;Piatek, Caroline I.;Brynes, Russell K.
自身免疫性骨髓纤维化（AIMF）是一种独特的临床病理实体与弥漫性骨髓纤维化和良性的临床过程。骨髓纤维化，特别是原发性骨髓纤维化的肿瘤病因的区别是必要的，但很少有研究记录了大量的组织病理学特征。我们描述了29例AIMF患者，定义为骨髓网硬蛋白纤维化和淋巴细胞浸润的背景下，一个既定的自身免疫性疾病（继发性AIMF）或自身抗体没有一个明确的疾病（原发性AIMF）。排除了非典型巨核细胞、发育异常、嗜碱性粒细胞增多、骨质增生、原因不明的脾肿大或骨髓纤维化（MF）相关肿瘤的病例。所有病例均进行了网硬蛋白、CD 3和CD 20染色，其中一个亚组还进行了CD 138、κ、λ、免疫球蛋白G（IgG）和IgG 4染色。如果存在类棘球蛋白聚集体，则将其分为T细胞和B细胞分布模式。大多数患者（93%）表现为血细胞减少。69%（n = 20）被认为是继发性AIMF，其余为原发性AIMF（n = 9）。外周血显示原始细胞和泪滴红细胞缺失至罕见，且无嗜酸性粒细胞增多或嗜碱性粒细胞增多。特征性骨髓检查结果包括红细胞和巨核细胞增生的细胞过多、轻度网硬蛋白纤维化、窦内造血、淋巴聚集体中的T细胞模式、轻度多型浆细胞增多和不存在IgG 4阳性浆细胞。原发性和继发性AIMF在病理学上难以区分，除了原发性AIMF中粒细胞增生不良的发生率增加。这一系列证实并扩展了AIMF原始诊断标准的实用性。认识到AIMF的特征性形态及其相关的临床和实验室特征将MF的自身免疫性与肿瘤性原因区分开来，并指导进一步的评估和管理。(C)2014爱思唯尔公司All rights reserved.
Autoimmune myelofibrosis (AIMF) is a distinct clinicopathological entity associated with diffuse bone marrow fibrosis and a benign clinical course. Distinction from neoplastic etiologies of marrow fibrosis, particularly primary myelofibrosis, is imperative, but few studies have documented histopathologic features in a large series. We describe 29 patients with AIMF, defined as marrow reticulin fibrosis and lymphocytic infiltration in the context of an established autoimmune disorder (secondary AIMF) or autoantibodies without a defined disorder (primary AIMF). Excluded were cases with atypical megakaryocytes, dysplasia, basophilia, osteosclerosis, unexplained splenomegaly, or neoplasms associated with myelofibrosis (MF). All cases were stained for reticulin, CD3, and CD20, with a subset additionally stained for CD138, kappa, lambda, immunoglobulin G (IgG), and IgG4. Lymphoid aggregates, where present, were classified into T-cell and B-cell patterns of distribution. Most patients (93%) presented with cytopenias. Sixty-nine percent (n = 20) were considered secondary AIMF and the remainder primary AIMF (n = 9). Peripheral blood showed absent-to-rare blasts and teardrop erythrocytes and absence of eosinophilia or basophilia. Characteristic bone marrow findings included hypercellularity with erythroid and megakaryocytic hyperplasias, mild reticulin fibrosis, intrasinusoidal hematopoiesis, T-cell pattern in lymphoid aggregates, mild polytypic plasmacytosis, and absence of IgG4-positive plasma cells. Primary and secondary AIMF were pathologically indistinguishable, except for an increased incidence of granulocytic hypeiplasia in primary AIM F. This series confirms and expands the utility of the original diagnostic criteria for AIMF. Recognizing the characteristic morphology of AIMF and its associated clinical and laboratory features distinguishes autoimmune from neoplastic causes of MF and guides further evaluation and management. (C) 2014 Elsevier Inc. All rights reserved.