Horny H P, Kaiserling E
Department of Special Histo- and Cytopathology, Eberhard-Karls University, Tübingen, F.R.G.
Br J Haematol. 1988 Aug;69(4):449-55. doi: 10.1111/j.1365-2141.1988.tb02397.x.
Systemic mastocytosis (SM) can be regarded as a tumorous proliferation of tissue mast cells (TMC) involving various organs, particularly the bone marrow. The infiltrates, however, are by no means composed exclusively of TMC, but also contain eosinophils and lymphocytes. The varying composition of the TMC infiltrates and the immunohistological characteristics of the lymphatic cells were the main subjects of investigation in this study. Three different types of bone marrow infiltrates could be identified: (1) A pure mastocytic infiltrate. (2) A mixed mastocytic/lymphocytic infiltrate. (3) A predominantly lymphocytic infiltrate containing loosely-scattered TMC. The mixed mastocytic/lymphocytic infiltrate seems to be a unique finding confined to the bone marrow in cases of SM, and is not detected in this conformation in other tissue sites normally involved in SM (spleen, liver, lymph nodes and skin), nor in cases of malignant mastocytosis. The lymphoid cells could be identified immunohistologically as being an admixture of T lymphocytes and B lymphocytes, while NK cells were virtually absent from the composite nodules. The TMC reacted strongly with antibodies (monoclonal or polyclonal) against vimentin, common leucocyte antigen, lysozyme, alpha 1-antichymotrypsin and alpha 1-antitrypsin, but were negative with a variety of other antibodies tested (UCHL1, MB1, Ki-B3, Leu-7, KL1, desmin, S-100 protein, F VIII-related antigen and chromogranin A).
系统性肥大细胞增多症(SM)可被视为组织肥大细胞(TMC)在包括骨髓在内的多个器官的肿瘤性增殖。然而,浸润细胞绝非仅由TMC组成,还包含嗜酸性粒细胞和淋巴细胞。TMC浸润的不同组成以及淋巴细胞的免疫组织学特征是本研究的主要调查对象。可识别出三种不同类型的骨髓浸润:(1)单纯肥大细胞浸润。(2)肥大细胞/淋巴细胞混合浸润。(3)以淋巴细胞为主的浸润,其中散在分布着少量TMC。肥大细胞/淋巴细胞混合浸润似乎是SM病例中骨髓特有的表现,在SM通常累及的其他组织部位(脾脏、肝脏、淋巴结和皮肤)以及恶性肥大细胞增多症病例中均未发现这种形态。免疫组织学上可确定淋巴样细胞是T淋巴细胞和B淋巴细胞的混合物,而复合结节中几乎不存在NK细胞。TMC与抗波形蛋白、共同白细胞抗原、溶菌酶、α1 -抗糜蛋白酶和α1 -抗胰蛋白酶的抗体(单克隆或多克隆)反应强烈,但对其他多种测试抗体(UCHL1、MB1、Ki - B3、Leu - 7、KL1、结蛋白、S - 100蛋白、F VIII相关抗原和嗜铬粒蛋白A)呈阴性反应。