Shimamura R, Kaneko T, Morioka E, Shibuya T, Ishibashi H, Niho Y
Rinsho Ketsueki. 1989 Jul;30(7):1067-73.
We treated 54-year-old Japanese man with a large cell type of Sézary syndrome. He had generalized erythrodermia, superficial lymphadenopathy, atypical lymphocytes in the peripheral blood, anti-HTLV-I antibody negativity and chromosomal abnormality. The patient was a hepatitis B virus carrier, and was complicated with hepatocellular carcinoma and monoclonal gammopathy of IgG, lambda type. Sézary syndrome is a T cell malignancy, the clinical course of which is relatively mild and chronic; accordingly, this case showed no crisis under chemotherapy. However, the patient died due to rapid growth of the hepatoma. Although case reports of Sézary syndrome complicated with other malignancies are very few, the occurrence of malignancies is possible because of decreased immunological function in the patients. In this case, hepatitis B virus might participate in the hepatic oncogenesis under dysfunction of helper/inducer cells. In addition, the complication of monoclonal gammopathy was also interesting from the standpoint of the helper function of Sézary cells.
我们治疗了一名54岁的日本男性,他患有大细胞型蕈样肉芽肿综合征。他有全身红皮病、浅表淋巴结病、外周血非典型淋巴细胞、抗HTLV-I抗体阴性及染色体异常。该患者是一名乙型肝炎病毒携带者,并合并肝细胞癌和IgG λ型单克隆丙种球蛋白病。蕈样肉芽肿综合征是一种T细胞恶性肿瘤,其临床病程相对较轻且呈慢性;因此,该病例在化疗下未出现危象。然而,患者因肝癌快速生长而死亡。虽然蕈样肉芽肿综合征合并其他恶性肿瘤的病例报告非常少,但由于患者免疫功能下降,发生恶性肿瘤是有可能的。在该病例中,乙型肝炎病毒可能在辅助/诱导细胞功能障碍的情况下参与肝脏肿瘤的发生。此外,从蕈样肉芽肿细胞的辅助功能角度来看,单克隆丙种球蛋白病的合并症也很有意思。