Beau P, Touchard G, Lessard M, Moreau C, Druart F, Morichau-Beauchant M, Tanzer J
Gastroenterol Clin Biol. 1984 Jan;8(1):57-61.
We report herein the case of a patient with Waldenström macroglobulinemia in whom an immunoblastic sarcoma produced acute jaundice and hepatic failure. Transvenous liver biopsy, performed because of coagulation deficiencies, revealed massive infiltration of the liver by the sarcoma. Immunofluorescence study showed numerous portal and sinusoidal cells stained exclusively by anti-micron anti-kappa antisera. As shown by others in the literature, this study supports evidence for the same clonal origin of the two proliferations. Chromosomal abnormalities were found in marrow and blood cells (trisomy 5 and 20, 6 q-, 7 q+, and 8 p-); their role in emergence of sarcoma is discussed. This case shows that immunoblastic sarcoma emerging during the course of Waldenström macroglobulinemia may be revealed by severe hepatic manifestations.
我们在此报告一例患有华氏巨球蛋白血症的患者，其免疫母细胞肉瘤导致了急性黄疸和肝衰竭。由于存在凝血功能缺陷而进行的经静脉肝活检显示，肉瘤大量浸润肝脏。免疫荧光研究表明，许多门静脉和窦状隙细胞仅被抗μ链抗κ轻链抗血清染色。正如文献中其他人所表明的那样，本研究支持这两种增殖具有相同克隆起源的证据。在骨髓和血细胞中发现了染色体异常（5号和20号染色体三体、6号染色体长臂缺失、7号染色体长臂增加和8号染色体短臂缺失）；讨论了它们在肉瘤发生中的作用。该病例表明，华氏巨球蛋白血症病程中出现的免疫母细胞肉瘤可能以严重的肝脏表现为首发症状。
