Kondo H, Yumoto N, Mikata A, Date Y
Division of Haematology and Oncology, Department of Medicine, Shimizu Kousei Hospital, Shizuoka, Japan.
Clin Mol Pathol. 1995 Aug;48(4):M215-8. doi: 10.1136/mp.48.4.m215.
A case of Waldenström's macroglobulinaemia with subsequent development of immunoblastic lymphoma in a 69 year old man is reported. Plasmacytoid lymphocytes were initially observed in both peripheral blood and bone marrow smears. Lymph node biopsy was interpreted as malignant lymphoma, diffuse mixed cell type compatible with Waldenström's macroglobulinaemia. Complete remission resulted on combined treatment with prednisolone and melphalan. Four years later, lymphadenopathy recurred and biopsy revealed the development of an immunoblastic lymphoma. Initially, this lymphoma was thought to be the malignant transformation of Waldenström's microglobulinaemia, because both tumours produced IgM (kappa light chains). Sequencing of immunoglobulin heavy chain genes ofWaldenström's macroglobulinaemia and immunoblastic lymphoma cells, however, revealed different Dh and Jh usage between the two, indicating the independent nature of the two haematological malignancies. These results indicate that clonality of primary and secondary tumours should be determined not by phenotypic, but by genotypic analysis.
报告了一例69岁男性的瓦尔登斯特伦巨球蛋白血症病例，该患者随后发展为免疫母细胞淋巴瘤。最初在外周血和骨髓涂片中均观察到浆细胞样淋巴细胞。淋巴结活检被解释为恶性淋巴瘤，弥漫性混合细胞型，符合瓦尔登斯特伦巨球蛋白血症。泼尼松龙和美法仑联合治疗后实现完全缓解。四年后，淋巴结病复发，活检显示发生了免疫母细胞淋巴瘤。最初，这种淋巴瘤被认为是瓦尔登斯特伦微球蛋白血症的恶性转化，因为两种肿瘤均产生IgM（κ轻链）。然而，对瓦尔登斯特伦巨球蛋白血症和免疫母细胞淋巴瘤细胞的免疫球蛋白重链基因进行测序后发现，两者之间的Dh和Jh使用情况不同，表明这两种血液系统恶性肿瘤具有独立性。这些结果表明，原发性和继发性肿瘤的克隆性不应通过表型分析，而应通过基因型分析来确定。
