Gebhart J, Lechner K, Skrabs C, Sliwa T, Müldür E, Ludwig H, Nösslinger T, Vanura K, Stamatopoulos K, Simonitsch-Klupp I, Chott A, Quehenberger P, Mitterbauer-Hohendanner G, Pabinger I, Jäger U, Geissler K
Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
5thDepartment of Medicine, Krankenhaus Hietzing, Vienna, Austria.
Thromb Res. 2014 Nov;134(5):980-4. doi: 10.1016/j.thromres.2014.08.021. Epub 2014 Aug 29.
Splenic marginal zone lymphoma (SMZL) is a rare low-malignant Non-Hodgkin lymphoma (NHL), in which immune mediated paraneoplastic phenomena such as autoimmune hemolytic anemia (AIHA), autoimmune thrombocytopenia (ITP) and C1 esterase inhibitor deficiency are relatively common.
We performed a multicenter retrospective study in 70 patients on the prevalence and clinical features of antiphospholipid antibodies (aPLA) in SMZL.
Nine patients (13%) had the diagnosis of a lupus anticoagulant (LA). The occurrence of venous thromboembolic events was significantly higher in LA positive patients compared to LA negative patients (4/9 [44%] vs 5/61 [8%], p = 0.002), especially within 12 months after splenectomy (3/6 [50%] vs 2/28 [7%], p = 0.007). None of the patients with LA had a persistent complete remission of LA after splenectomy, but complete remission of LA was achieved in 2/2 patients after rituximab-bendamustine immuno-chemotherapy. In conclusion, our data show a relatively high prevalence of aPLA in SMZL and an increased risk of postsplenectomy thrombosis in these patients. The fact that rituximab-bendamustine was effective for eradicating LA may be considered as an argument for using immuno-chemotherapy as first line therapy in SMZL patients with LA.
脾边缘区淋巴瘤（SMZL）是一种罕见的低恶性非霍奇金淋巴瘤（NHL），其中免疫介导的副肿瘤现象如自身免疫性溶血性贫血（AIHA）、自身免疫性血小板减少症（ITP）和C1酯酶抑制剂缺乏相对常见。
我们对70例患者进行了一项多中心回顾性研究，以探讨脾边缘区淋巴瘤中抗磷脂抗体（aPLA）的患病率及临床特征。
9例患者（13%）诊断为狼疮抗凝物（LA）。与LA阴性患者相比，LA阳性患者静脉血栓栓塞事件的发生率显著更高（4/9 [44%] 对5/61 [8%]，p = 0.002），尤其是在脾切除术后12个月内（3/6 [50%] 对2/28 [7%]，p = 0.007）。LA患者脾切除术后均未实现LA的持续完全缓解，但利妥昔单抗-苯达莫司汀免疫化疗后2/2例患者实现了LA的完全缓解。总之，我们的数据显示脾边缘区淋巴瘤中aPLA的患病率相对较高，且这些患者脾切除术后血栓形成风险增加。利妥昔单抗-苯达莫司汀对根除LA有效这一事实可被视为在伴有LA的脾边缘区淋巴瘤患者中使用免疫化疗作为一线治疗的一个依据。
