华氏巨球蛋白血症的免疫表型分析。

Immunophenotypic analysis of Waldenstrom's macroglobulinemia.

作者信息

San Miguel J F, Vidriales M B, Ocio E, Mateo G, Sánchez-Guijo F, Sánchez M L, Escribano L, Bárez A, Moro M J, Hernández J, Aguilera C, Cuello R, García-Frade J, López R, Portero J, Orfao A

机构信息

Servicio de Hematología, Hospital Universitario de Salamanca, Spain.

出版信息

Semin Oncol. 2003 Apr;30(2):187-95. doi: 10.1053/sonc.2003.50074.

DOI:10.1053/sonc.2003.50074

PMID:12720134

Abstract

Immunophenotyping has become an essential tool for diagnosis of hematological malignancies. By contrast, for diagnosis of Waldenstrom's macroglobulinemia (WM) immunophenotyping is used only occasionally. From 150 patients with a IgM monoclonal gammopathy we have selected 60 cases with (1) morphological lymphoplasmocytoid bone marrow (BM) infiltration (>20%); (2) IgM paraprotein (>10g/L); and (3) absence of features of other lymphoma types. Immunophenotypic analysis was based on the use of the triple or quadruple monoclonal antibody (MoAb) combinations. To increase the sensitivity of the analysis of antigen expression, selected CD19(+)CD20(+) B cells were targeted. We have also explored the antigenic characteristics of both the plasma cell (PC) and mast cell (MC) compartments present in the BM from 15 WM patients. Clonal WM lymphocytes were characterized by the constant expression of pan-B markers (CD19, CD20, CD22, CD24) together with sIg, predominantly kappa (5:1, kappa:lambda ratio). A high proportion of cases (75%) were positive for FMC7 and CD25, but in contrast to hairy cell leukemia (HCL), these lymphocytes were always negative for CD103 and CD11c. CD10 antigen was also absent in all WM patients and less than one fifth of patients were positive for CD5 and CD23, while CD27, CD45RA, and BCL-2 were present in most malignant cells. In two cases, the coexistence of two different clones of B lymphocytes was identified, and in eight additional cases, intraclonal phenotypic heterogeneity was observed. As far as PCs are concerned, in most patients (85%) the number of PCs was within the normal range (median, 0.36%). The antigenic profile of these PCs differed from that observed in normal and myelomatous PC (CD38(++)CD19(++/-)CD56(-)CD45(++)CD20(+)). In three cases, PCs showed aberrant expression for CD5, CD22, or FMC7. Finally, the number of mast cells was significantly higher (0.058 +/- 0.13) as compared to normal BM (0.019 +/- 0.02) (P <.01), although they were immunophenotypically normal (CD117(+)CD2(-)CD25(-)).

摘要

免疫表型分析已成为诊断血液系统恶性肿瘤的重要工具。相比之下，免疫表型分析仅偶尔用于诊断华氏巨球蛋白血症（WM）。从150例患有IgM单克隆丙种球蛋白病的患者中，我们选取了60例具有以下特征的病例：（1）形态学上淋巴浆细胞样骨髓（BM）浸润（>20%）；（2）IgM副蛋白（>10g/L）；（3）无其他淋巴瘤类型的特征。免疫表型分析基于使用三联或四联单克隆抗体（MoAb）组合。为了提高抗原表达分析的敏感性，选取CD19(+)CD20(+) B细胞作为目标。我们还研究了15例WM患者BM中浆细胞（PC）和肥大细胞（MC）区室的抗原特征。克隆性WM淋巴细胞的特征是泛B标志物（CD19、CD20、CD22、CD24）与sIg持续表达，主要为kappa（kappa:lambda比例为5:1）。高比例病例（75%）FMC7和CD25呈阳性，但与毛细胞白血病（HCL）不同，这些淋巴细胞CD103和CD11c始终为阴性。所有WM患者CD10抗原也均缺失，不到五分之一的患者CD5和CD23呈阳性，而大多数恶性细胞中存在CD27、CD45RA和BCL-2。在2例病例中，鉴定出两种不同B淋巴细胞克隆共存，另外8例病例中观察到克隆内表型异质性。就PC而言，大多数患者（85%）PC数量在正常范围内（中位数为0.36%）。这些PC的抗原谱与正常和骨髓瘤PC中观察到的不同（CD38(++)CD19(++/-)CD56(-)CD45(++)CD20(+)）。在3例病例中，PC显示CD5、CD22或FMC7表达异常。最后，肥大细胞数量显著高于正常BM（0.058±0.13 vs 0.019±0.02）（P<.01），尽管其免疫表型正常（CD117(+)CD2(-)CD25(-)）。