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华氏巨球蛋白血症的骨髓组织学。亚型识别的临床意义。

Bone marrow histology in Waldenström's macroglobulinaemia. Clinical relevance of subtype recognition.

作者信息

Bartl R, Frisch B, Mahl G, Burkhardt R, Fateh-Moghadam A, Pappenberger R, Sommerfeld W, Hoffmann-Fezer G

出版信息

Scand J Haematol. 1983 Oct;31(4):359-75. doi: 10.1111/j.1600-0609.1983.tb00664.x.

Abstract

Bone marrow biopsies of 137 patients with Waldenström's macroglobulinaemia (WM), 26 with non-secretory immunocytoma and 32 with benign monoclonal gammopathy were processed for histologic evaluation. Bone marrow involvement was found in 110 (80%) initially, and in 24 (18%) in sequential biopsies. 3 types were distinguished: lymphoplasmacytoid (47%), lymphoplasmacytic (42%) and polymorphous (11%) with median survivals of 74, 25 and 12 months, respectively. When grouped according to the tumour cell mass in the biopsies, the median survivals were 55, 21 and 8 months for less than 20 vol%, 20-50 vol% and greater than 50 vol% respectively; in each subtype, the tumour cell mass correlated with the disease progression. 6 clinical variables were also found prognostically significant. These results demonstrate that (i) 98% of patients with WM have bone marrow involvement; (ii) the lymph node sub-classification is applicable to the bone marrow and has both clinical and prognostic significance; (iii) patients may be staged according to the tumour cell burden in the bone marrow biopsy.

摘要

对137例华氏巨球蛋白血症(WM)患者、26例非分泌性免疫细胞瘤患者和32例良性单克隆丙种球蛋白病患者的骨髓活检标本进行组织学评估。最初发现110例(80%)有骨髓受累,后续活检发现24例(18%)有骨髓受累。区分出3种类型:淋巴浆细胞样(47%)、淋巴浆细胞性(42%)和多形性(11%),中位生存期分别为74、25和12个月。根据活检中肿瘤细胞团块分组,肿瘤细胞团块小于20%体积、20%-50%体积和大于50%体积时的中位生存期分别为55、21和8个月;在每个亚型中,肿瘤细胞团块与疾病进展相关。还发现6个临床变量具有预后意义。这些结果表明:(i)98%的WM患者有骨髓受累;(ii)淋巴结分类适用于骨髓,具有临床和预后意义;(iii)可根据骨髓活检中的肿瘤细胞负荷对患者进行分期。

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