Intralymphatic Histiocytosis. A Clinicopathologic Study of 16 Cases
Intralymphatic Histiocytosis. A Clinicopathologic Study of 16 Cases
复制标题
DOI：
10.1097/dad.0b013e3181986cc2
复制
发表时间：
2009-04-01
影响因子：
1.1
通讯作者：
Kutzner, Heinz
中科院分区：
文献类型：
--
作者：
Requena, Luis;El-Shabrawi-Caelen, Laila;Kutzner, Heinz
淋巴内组织细胞增生症是一种罕见的疾病，其特征是存在扩张的淋巴管，其管腔内含有单核组织细胞（巨噬细胞）的聚集体。这种现象似乎几乎只发生在网状真皮内。虽然其发病机制仍然不确定，有关于淋巴管内组织细胞增生症和血管内反应性血管内皮瘤病之间的可能关系的猜测。此外，历史上有几个例子与类风湿性关节炎有关。我们描述我们的经验与16例淋巴内组织细胞增生症。临床上，病变主要位于上肢和下肢，包括无症状和边界不清的斑块和青斑网状病变。其组织病理学特征为累及网状真皮的扩张血管结构。这些扩张的血管中有一些管腔是空的，而另一些则含有数量不等的单核组织细胞。在相邻真皮中也存在不同强度的炎症反应。扩张的血管壁薄，形状不规则，管腔内排列着一层不连续的扁平内皮细胞。免疫组化显示，扩张管腔内的内皮细胞表达CD 31、CD 34、podoplanin、D2-40、Lyve-1和Prox-1，这证实了它们作为淋巴管内皮细胞的性质。淋巴管内单核组织细胞表达CD 68（PGM 1），虽然有些情况下也有髓过氧化物酶，CD 31和podoplanin的免疫表达。在4例采用双重免疫组化的病例中，podoplanin + CD 68（PGM 1）或Lyve-1 + CD 68（PGM 1），每种标记物均明确突出其特异性靶细胞;内皮细胞表达podoplanin或Lyve-1免疫反应性，淋巴管内组织细胞显示CD 68（P（M）免疫反应性。我们的发现扩展了以前描述的血管内组织细胞增生症的形态学和免疫组化特征。我们还讨论了淋巴内组织细胞增生症和所谓的反应性血管内血管内皮瘤病之间的可能关系。
Intralymphatic histiocytosis is a rare condition characterized by the presence of dilated lymphatic vessels containing aggregates of mononuclear histiocytes (macrophages) within their lumina. The phenomenon seems to occur almost exclusively within the reticular dermis. Although its pathogenesis remains uncertain, there has been speculation about the possible relationship between intralymphatic histiocytosis and intravascular reactive angioendotheliomatosis. In addition, several examples historically have been associated with rheumatoid arthritis. We describe our experience with 16 cases of intralymphatic histiocytosis. Clinically, the lesions were located predominantly on the upper and lower limbs, and they consisted of asymptomatic and poorly demarcated erythematous plaques and livedo reticularis-like lesions. They were characterized histopathologically by dilated vascular structures involving the reticular dermis. Some of these dilated vessels had empty lumina, whereas others contained variable number of mononuclear histiocytes. An inflammatory response of variable intensity from case to case was also present in the adjacent dermis. The dilated vessels exhibited thin walls with irregular shapes, and a single discontinuous layer of flat endothelial cells lined their lumina. Immunohistochemically, the endothelial cells lining the dilated lumina expressed immunoreactivity for CD31, CD34, podoplanin, D2-40, Lyve-1, and Prox-1, which confirmed their nature as lymphatic endothelial cells. Intralymphatic mononuclear histiocytes expressed CD68 (PGM1), although some cases also had variable immunoexpression for myeloperoxidase, CD31, and podoplanin. In the 4 cases that employed double immunohistochemistry, with podoplanin + CD68 (PGM1) or with Lyve-1 + CD68 (PGM1), each marker highlighted their specific target cells unequivocally; the endothelial cells expressed podoplanin or Lyve-1 immunoreactivity, and intralymphatic histiocytes showed CD68 (P(M) immunoexpression. Our findings expand on the previously described morphologic and immunohistochemical features of intravascular histiocytosis. We also discuss the possible relationship between intralymphatic histiocytosis and the so-called reactive intravascular angioendotheliomatosis.