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[浆细胞瘤和华氏巨球蛋白血症的诊断与治疗]

[Diagnosis and therapy of plasmacytoma and Waldenstrom's disease].

作者信息

Kühn R A

出版信息

Z Gesamte Inn Med. 1976 Apr 15;31(8):244-50.

PMID:822613
Abstract

Plasmocytoma and Waldenstroem's disease together with some other very rare diseases form the group of paraproteinaemic haemoblastoses. In these cases the plasmocytoma as a neoplasia of the plasma cells is quite likely to define by the sum of the cytomorphological, radiological and immunological findings in form of the plasmocytosis of the bone-marrow, the destructions of the skeleton and the formation of monoclonal immunoglobulins as cardinal symptoms. Waldenstroem's disease, however, becomes more and more a special form of the IgM-forming malignant lymphomas. In its classical expression apart from the formation of lymphomas, hepato-splenomegaly and haemorhages it above all exhibits the excessive formation of a monoclonal, macromolecular immunoglobulin. In this disease the prognosis is in many cases by far more favourable than that one of the plasmocytoma which, apart from its solitary-multiple form of the course, is a disease with high malignity. Cyclophosphamide and sarcolysin are the remedies of choice in the plasmocytoma, procarbazine in waldenstroem's disease. Nowadays, the inclusion of the two diseases into the large spectre of neoplasias of all cells of the immuno-competent system which finally are structurally derived from the immunocyte and are functionally differentiated renders a special way of description unnecessary.

摘要

浆细胞瘤和华氏巨球蛋白血症以及其他一些非常罕见的疾病构成了副蛋白血症性成血细胞增生症组。在这些病例中,浆细胞瘤作为浆细胞的一种肿瘤,很可能由骨髓浆细胞增多、骨骼破坏以及单克隆免疫球蛋白形成等细胞形态学、放射学和免疫学检查结果综合确定为主要症状。然而,华氏巨球蛋白血症越来越成为形成IgM的恶性淋巴瘤的一种特殊形式。在其典型表现中,除了形成淋巴瘤、肝脾肿大和出血外,它首先表现为单克隆大分子免疫球蛋白的过度形成。在这种疾病中,许多情况下预后远比浆细胞瘤要好,浆细胞瘤除了其病程的单发-多发形式外,是一种高度恶性的疾病。环磷酰胺和溶肉瘤素是浆细胞瘤的首选治疗药物,甲基苄肼用于治疗华氏巨球蛋白血症。如今,将这两种疾病纳入免疫活性系统所有细胞的肿瘤大范畴,这些细胞最终在结构上源自免疫细胞并在功能上分化,使得无需采用特殊的描述方式。

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