Gross T G, Steinbuch M, DeFor T, Shapiro R S, McGlave P, Ramsay N K, Wagner J E, Filipovich A H
Department of Pediatrics, University of Minnesota Hospital and Clinics, Minneapolis, USA.
Bone Marrow Transplant. 1999 Feb;23(3):251-8. doi: 10.1038/sj.bmt.1701554.
Twenty-six cases of B cell lymphoproliferative disorder (BLPD) were identified among 2395 patients following hematopoietic stem cell transplants (HSCT) for which an overall incidence of BLPD was 1.2%. The true incidence was probably higher, since 9/26 of the diagnoses were made at autopsy. No BLPD was observed following autologous HSCT, so risk factor analyses were confined to the 1542 allogeneic HSCT. Factors assessed were HLA-mismatching (> or = 1 antigen), T cell depletion (TCD), presence of acute GvHD (grades II-IV), donor type (related vs unrelated), age of recipient and donor, and underlying disease. Factors found to be statistically significant included patients transplanted for immune deficiency and CML, donor age > or = 18 years, TCD, and HLA-mismatching, with recipients of combined TCD and HLA-mismatched grafts having the highest incidence. Factors found to be statistically significant in a multiple regression analysis were TCD, donor age and immune deficiency, although 7/8 of the patients with immunodeficiencies and BLPD received a TCD graft from a haploidentical parent. The overall mortality was 92% (24/26). One patient had a spontaneous remission, but subsequently died >1 year later of chronic GVHD. Thirteen patients received therapy for BLPD. Three patients received lymphocyte infusions without response. The only patients with responses and longterm survival received alpha interferon (alphaIFN). Of seven patients treated with alphaIFN there were four responses (one partial and three complete). These data demonstrate that alphaIFN can be an effective agent against BLPD following HSCT, if a timely diagnosis is made.
在2395例造血干细胞移植（HSCT）患者中，确诊了26例B细胞淋巴增殖性疾病（BLPD），BLPD的总体发病率为1.2%。由于26例中有9例诊断是在尸检时做出的，所以实际发病率可能更高。自体HSCT后未观察到BLPD，因此风险因素分析仅限于1542例异基因HSCT。评估的因素包括HLA错配（≥1个抗原）、T细胞去除（TCD）、急性移植物抗宿主病（II-IV级）的存在、供体类型（亲属与非亲属）、受者和供者的年龄以及基础疾病。发现具有统计学意义的因素包括因免疫缺陷和慢性粒细胞白血病而接受移植的患者、供体年龄≥18岁、TCD和HLA错配，接受TCD与HLA错配联合移植的受者发病率最高。在多元回归分析中发现具有统计学意义的因素是TCD、供体年龄和免疫缺陷，尽管7/8患有免疫缺陷和BLPD的患者接受了来自单倍体相合亲本的TCD移植。总体死亡率为92%（24/26）。1例患者自发缓解，但随后在1年多后死于慢性移植物抗宿主病。13例患者接受了BLPD治疗。3例患者接受淋巴细胞输注但无反应。唯一有反应并长期存活的患者接受了α干扰素（αIFN）治疗。在接受αIFN治疗的7例患者中有4例有反应（1例部分缓解，3例完全缓解）。这些数据表明，如果能及时诊断，αIFN可以成为HSCT后治疗BLPD的有效药物。
