Umeki S, Shibayama T, Izumi K, Sezaki T
Rinsho Ketsueki. 1989 Jan;30(1):89-93.
A 88-year-old man was admitted because of the left chest pain due to herpes zoster for 1 week. Blood analyses and immunoelectrophoresis revealed anemia, severe neutropenia, rouleaux formation and IgM, lambda-type monoclonal gammopathy. The HE staining and peroxidase-anti-peroxidase staining of biopsy specimens of the cervical lymph node swelling appeared from the fifth hospital day, revealed an increase in atypical lymphocytes bearing IgM, lambda-type immunoglobulin. Then a diagnosis of primary macroglobulinemia was made. Although the patient's clinical findings transiently improved after chemotherapy with prednisolone and vindesine, he died of a septic shock which appeared after klebsiella pneumonia and sepsis. We reported an unusual case of primary macroglobulinemia with severe neutropenia, leading to a rapid development of septic shock after the chemotherapy.
一名88岁男性因带状疱疹导致左胸痛1周入院。血液分析和免疫电泳显示贫血、严重中性粒细胞减少、红细胞缗钱状形成以及IgM、λ型单克隆丙种球蛋白病。自住院第5天起，颈部肿大淋巴结活检标本的苏木精-伊红染色和过氧化物酶-抗过氧化物酶染色显示，带有IgM、λ型免疫球蛋白的非典型淋巴细胞增多。随后诊断为原发性巨球蛋白血症。尽管患者在接受泼尼松龙和长春地辛化疗后临床症状暂时改善，但他死于肺炎克雷伯菌肺炎和败血症后出现的感染性休克。我们报告了一例罕见的原发性巨球蛋白血症病例，伴有严重中性粒细胞减少，化疗后导致感染性休克迅速发展。
