Wick M R, Rocamora A
Department of Pathology, University of Minnesota School of Medicine, Minneapolis.
J Cutan Pathol. 1988 Oct;15(5):260-71. doi: 10.1111/j.1600-0560.1988.tb00557.x.
In order to determine whether or not phenotypic differences existed between reactive angioendotheliomatosis (RAE) and malignant angioendotheliomatosis (MAE), we studied the histological and immunohistochemical features of 4 and 8 cases of these lesions, respectively. Antibodies to leukocyte common antigen (LCA), specialized B- and T-lymphocytic determinants, Factor VIII-related antigen (FVIIIRAG), blood group isoantigens A, B, and H (BGI), epithelial antigens, vimentin, and actin; and Ulex europaeus I lectin (UEL) were utilized. Cutaneous lesions in all cases of MAE were part of a disseminated, fatal, intravascular cellular proliferation, with highly atypical cytological features. Because one of the patients in this group had cardiac valvular vegetations at autopsy, this case had been reported previously as representative of RAE. However, the latter example, as well as all others of MAE, stained strongly for LCA, B-cell antigens, and vimentin in tumor cells. FVIIIRAG was seen focally in 6 cases, in cells entrapped in platelet-fibrin thrombi; however, UEL binding and reactivity for BGI were uniformly absent. Conversely, RAE was typified by a cytologically-bland intravascular proliferation, with actin-positive, perivascular, pericytic cuffs. All 4 patients in this group had cutaneous involvement only, and the lesions tended to be self-resolving. One had pulmonary tuberculosis, but evidence for an underlying infection was absent in the remainder of RAE cases. Immunohistologically, RAE displayed universal reactivity for FVIII-RAG, BGI, UEL, and vimentin, and negativity for LCA in intravascular cells. Neither MAE nor RAE showed the presence of epithelial determinants. These data indicate that MAE and RAE are clinicopathologically distinct entities, showing lymphoid and endothelial features, respectively. Because of the phenotypic properties of the former condition, it would appear advisable to substitute the term "intravascular lymphomatosis" for "malignant angioendotheliomatosis".
为了确定反应性血管内皮瘤病（RAE）与恶性血管内皮瘤病（MAE）之间是否存在表型差异，我们分别研究了4例RAE和8例MAE病变的组织学和免疫组化特征。使用了针对白细胞共同抗原（LCA）、特异性B和T淋巴细胞决定簇、因子VIII相关抗原（FVIIIRAG）、血型同种抗原A、B和H（BGI）、上皮抗原、波形蛋白和肌动蛋白的抗体；以及荆豆凝集素I（UEL）。所有MAE病例的皮肤病变都是播散性、致命性血管内细胞增殖的一部分，具有高度非典型的细胞学特征。因为该组中的一名患者在尸检时有心脏瓣膜赘生物，该病例先前已作为RAE的代表被报道。然而，后一个例子以及所有其他MAE病例，肿瘤细胞中LCA、B细胞抗原和波形蛋白染色均呈强阳性。FVIIIRAG在6例中局灶性可见，存在于血小板 - 纤维蛋白血栓中包绕的细胞内；然而，UEL结合和对BGI的反应性均未出现。相反，RAE的特征是细胞学上温和的血管内增殖，伴有肌动蛋白阳性的血管周围、周细胞套。该组的所有4例患者仅累及皮肤，病变倾向于自行消退。1例患有肺结核，但其余RAE病例中没有潜在感染的证据。免疫组化方面，RAE在血管内细胞中对FVIII - RAG、BGI、UEL和波形蛋白呈普遍反应性，对LCA呈阴性。MAE和RAE均未显示上皮决定簇的存在。这些数据表明MAE和RAE是临床病理上不同的实体，分别表现出淋巴样和内皮样特征。由于前一种情况的表型特性，用“血管内淋巴瘤病”取代“恶性血管内皮瘤病”这一术语似乎是可取的。
