Akimoto Y, Ishiyama T, Kawakami K, Hino K, Tomoyasu S, Tsuruoka N, Kushima M, Ota S, Kazama K
Department of Hematology, Showa University School of Medicine.
Rinsho Ketsueki. 1995 Aug;36(8):742-8.
A 49-year-old man was admitted to our hospital with anemia and hypergammaglobulinemia. Physical examination revealed superficial lymph node swelling and no hepatosplenomegaly. Laboratory findings showed WBC 5,300/microliters with normal hemogram, microcytic and hypochromic anemia. Total protein was 11.5 g/dl and immunoglobulinemia (IgG 10,100 mg/dl, IgA 295 mg/dl, IgM 160 mg/dl) was observed without M-component in serum and urine. The CD4/CD8 ratio of lymphocyte subsets was 0.58 and the tuberuculin skin test was negative. Urinary protein was positive and renal biopsy disclosed plasma cell infiltration. Lymph node biopsy revealed multiple lymphoid follicles and infiltration of plasma cells in the interfollicular areas. A diagnosis of multicentric Castleman's disease (MCD) was made baredon clinical findings and lymph node biopsy. After therapy with plasmapheresis and the CHOP regimen, he was given etoposide. Although discharged with clinical improvement and a decrease of serum IgG, he was readmitted because of pyrexia after 4 days and died of pneumonia with adult respiratory distress syndrome. The autopsy revealed lymphoid interstitial pneumonia. It seems important to notice that some of MCD have poor prognoses because of accompanying immunodeficiency.
一名49岁男性因贫血和高球蛋白血症入住我院。体格检查发现浅表淋巴结肿大，无肝脾肿大。实验室检查结果显示白细胞计数为5300/微升，血常规正常，为小细胞低色素性贫血。总蛋白为11.5g/dl，血清和尿液中观察到免疫球蛋白血症（IgG 10100mg/dl，IgA 295mg/dl，IgM 160mg/dl），无M成分。淋巴细胞亚群的CD4/CD8比值为0.58，结核菌素皮肤试验为阴性。尿蛋白阳性，肾活检显示浆细胞浸润。淋巴结活检显示多个淋巴滤泡，滤泡间区有浆细胞浸润。根据临床表现和淋巴结活检结果诊断为多中心Castleman病（MCD）。在进行血浆置换和CHOP方案治疗后，给予其依托泊苷。尽管出院时临床症状改善，血清IgG降低，但4天后因发热再次入院，死于伴有成人呼吸窘迫综合征的肺炎。尸检显示为淋巴间质性肺炎。需要注意的是，部分MCD患者由于伴有免疫缺陷，预后较差。
