Lindahl J, Kimby E, Björkstrand B, Christensson B, Hellström-Lindberg E
Department of Medicine, Division of Hematology, Karolinska Institutet, Huddinge University Hospital, 141 86 Stockholm, Sweden.
Leuk Res. 2001 Mar;25(3):267-70. doi: 10.1016/s0145-2126(00)00134-x.
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.
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。
