1st Dept. of Internal Medicine, Faculty of Medicine, Semmelweis University, Koranyi Sandor utca 2/A, Budapest, Hungary, H-1083.
Pathol Oncol Res. 2009 Dec;15(4):597-603. doi: 10.1007/s12253-009-9159-8. Epub 2009 Apr 3.
Splenic marginal zone lymphoma is a rare disease, accounting for 1% of all lymphomas. We reviewed our single center experience of 13 patients with splenic marginal zone lymphoma (SMZL). Based on the prognostic model developed by Intergruppo Italiano Linfomi, 31% (4/13) of our patients had good, 38% (5/13) had intermediate and 31% (4/13) had a poor prognosis. The presence of two out of three prognostic factors (anemia, elevated LDH, low serum albumin) assignes the patient into the high risk category. In patients with anemia and an elevated LDH due to hemolysis, the outcome seems to be especially poor. Three out of 13 (23%) cases were complicated by autoimmune hemolytic anemia. All patients with autoimmune hemolytic anaemia (AIHA) died 7-28 months after the diagnosis. The mean follow-up time of those nine patients who are still alive is longer than 5 years (36-100 months). Patients with AIHA had significantly (p < 0.001) worse survival than those without AIHA. The main finding of our study is that the presence of AIHA is an adverse prognostic factor in SMZL.
脾边缘区淋巴瘤是一种罕见疾病，约占所有淋巴瘤的 1%。我们回顾了我们单中心的 13 例脾边缘区淋巴瘤（SMZL）患者的经验。根据意大利淋巴瘤研究组制定的预后模型，我们的患者中有 31%（4/13）预后良好，38%（5/13）预后中等，31%（4/13）预后较差。有两种（贫血、LDH 升高、血清白蛋白降低）预后因素的患者归入高危类别。在因溶血而出现贫血和 LDH 升高的患者中，预后似乎特别差。13 例患者中有 3 例（23%）合并自身免疫性溶血性贫血。所有自身免疫性溶血性贫血（AIHA）患者均在诊断后 7-28 个月死亡。9 例仍存活患者的平均随访时间超过 5 年（36-100 个月）。有 AIHA 的患者的生存明显（p < 0.001）差于无 AIHA 的患者。我们研究的主要发现是 AIHA 的存在是 SMZL 的一个不良预后因素。
