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大剂量化疗及自体外周血干细胞移植作为复发性溶血性自身免疫性淋巴细胞增多症的一种潜在治愈方法。

High-dose chemotherapy and APSCT as a potential cure for relapsing hemolysing AILD.

作者信息

Lindahl J, Kimby E, Björkstrand B, Christensson B, Hellström-Lindberg E

机构信息

Department of Medicine, Division of Hematology, Karolinska Institutet, Huddinge University Hospital, 141 86 Stockholm, Sweden.

出版信息

Leuk Res. 2001 Mar;25(3):267-70. doi: 10.1016/s0145-2126(00)00134-x.

Abstract

Angioimmunoblastic lymphadenopathy with dysproteinemia (or dysgammaglobulinemia) (AILD) is a lymphoproliferative disorder with cytogenetic and molecular abnormalities characteristic of malignant T-cell lymphoma (angioimmunoblastic T-cell lymphoma -- AITL). We report the clinical course of a 58-year-old male patient with unusually aggressive AILD, including severe hemolysis and Guillain-Barré syndrome, who entered complete remission after CHOP therapy, but had a full relapse after 2 months. At relapse, treatment with high-dose chemotherapy followed by autologous peripheral stem cell transplantation (APSCT) with CD34 selected cells was shown to be successful. The patient is alive and disease-free 3 years after diagnosis and 32 months after APSCT. Considering the poor prognosis of the majority of patients with AILD, intensive treatment followed by APSCT, may be a subject for further studies.

摘要

血管免疫母细胞性淋巴结病伴蛋白异常血症(或γ球蛋白异常血症)(AILD)是一种具有恶性T细胞淋巴瘤(血管免疫母细胞性T细胞淋巴瘤——AITL)特征性细胞遗传学和分子异常的淋巴增殖性疾病。我们报告了一名58岁男性患者的临床病程,该患者患有异常侵袭性AILD,包括严重溶血和吉兰 - 巴雷综合征,其在CHOP治疗后进入完全缓解期,但2个月后完全复发。复发时,采用高剂量化疗后行CD34选择细胞的自体外周血干细胞移植(APSCT)治疗显示成功。该患者在诊断后3年及APSCT后32个月仍存活且无疾病。考虑到大多数AILD患者预后较差,高强度治疗后行APSCT可能是进一步研究的课题。

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