Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation
Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation
复制标题
反应性血管内皮瘤病或血管内组织细胞增多症？
DOI：
10.1046/j.1365-2133.1999.02717.x
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发表时间：
1999
影响因子：
10.3
通讯作者：
H. Kerl
中科院分区：
文献类型：
--
作者：
E. Rieger;H. Soyer;P. Leboit;D. Metze;R. Slovak;H. Kerl
两名有复杂病史的老年妇女出现了多发性斑块，一例涉及面部和前臂，另一例涉及两个肘部。2例患者的打孔活检显示中等大小和大细胞的血管内增殖，伴血管内皮瘤病典型的管腔闭塞。免疫染色未显示淋巴细胞或内皮细胞抗原，但在这两种情况下，血管内细胞的组织细胞分化是一致的，并进一步证实了超微结构检查在一个案件。1例患者接受了超过15天的环磷酰胺治疗。皮肤病变消退但未消失。10个月后，患者死于心脏和肾衰竭，这很可能与皮肤病变无关。在另一种情况下，病变缩小，但没有完全解决与低剂量的口服泼尼松治疗。血管内皮瘤病可分为恶性变体和良性反应性变体，恶性变体是一种主要为B-细胞表型的亲血管性淋巴瘤，良性反应性变体的特征是表达内皮细胞标志物的细胞增殖。只有一例血管内皮细胞瘤病与组织细胞分化的细胞已发表以前的名称血管内组织细胞增生症。我们的情况与后者非常相似。问题是血管内组织细胞增殖是组织细胞的肿瘤性增殖还是典型反应性血管内皮瘤病的早期阶段，代表与微血栓组织化相关的残留细胞，随后是内皮细胞增殖。
Two elderly women with complex medical histories presented with erythematous patches, in one case involving the face and forearms, and in the other both elbows. Punch biopsies from both patients revealed intravascular proliferations of medium‐sized and large cells with luminal occlusion typical of angioendotheliomatosis. Immunostaining did not show either lymphocytic or endothelial cell antigens but was consistent with a histiocytic differentiation of the intravascular cells in both cases, and was further substantiated by ultrastructural examination in one case. One patient received a course of cyclophosphamide therapy over 15 days. Skin lesions faded but did not disappear. The patient died 10 months later from cardiac and renal failure, which was most probably unrelated to the skin lesions. In the other case, lesions diminished but did not entirely resolve with treatment with low doses of oral prednisone. Angioendotheliomatosis can be divided into a malignant variant, which is an angiotropic lymphoma mostly of B‐cell phenotype, and a benign, reactive variant, which is characterized by a proliferation of cells expressing endothelial cell markers. Only one case of angioendotheliomatosis with cells of histiocytic differentiation has been published previously under the name of intravascular histiocytosis. Our cases are very similar to the latter. The question arises as to whether intravascular histiocytic cell proliferation is a neoplastic proliferation of histiocytes or an early stage of classic reactive angioendotheliomatosis representing the residual cells associated with organization of microthrombi, which will be later followed by endothelial cell proliferation.