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在一家三级保健中心患有系统性红斑狼疮和血液系统恶性肿瘤的患者:时间、组织病理学和治疗。

Patients with systemic lupus erythematosus and haematological malignancy at a tertiary care centre: timing, histopathology and therapy.

机构信息

Department of Internal Medicine, Division of Rheumatology , University of Michigan , Ann Arbor, Michigan , USA.

Stanford Cancer Center, Stanford School of Medicine , Stanford, California , USA.

出版信息

Lupus Sci Med. 2014 Nov 14;1(1):e000051. doi: 10.1136/lupus-2014-000051. eCollection 2014.

Abstract

OBJECTIVES

Patients with systemic lupus erythematosus (SLE) are at higher risk of haematological malignancies (HMs) than the general population. Most reports have focused on HM diagnosed after SLE, and have excluded concurrent and preceding diagnoses. Information on response to therapy is also limited.

METHODS

We identified 13 296 cases of HM and 10 539 potential patients with SLE at our centre; 45 patients were confirmed to have HM and SLE. Our retrospective case series was based on these 45 patients.

RESULTS

Of the 45 patients, 64% were diagnosed with HM ≥1 year after diagnosis with SLE, and 36% with HM before or concurrent with SLE. Of the 29 patients with HM after SLE, 13 had diffuse large B cell lymphoma (DLBCL), 6 indolent lymphoma, 4 leukaemia, 3 Hodgkin's disease, and 1 each Burkitt's lymphoma, T cell lymphoma and multiple myeloma. Eleven patients with DLBCL were treated with cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone (CHOP) or rituximab-CHOP; hydroxydaunorubicin, oncovin and prednisone; only four achieved durable remission. Of the 16 patients diagnosed with HM before or concurrent with SLE, 9 were diagnosed with HM more than 2 years before SLE and tended to be in remission prior to SLE diagnosis. Seven patients were diagnosed with HM and SLE concurrently; in terms of their HM, six achieved remission or stable disease.

CONCLUSIONS

In summary, DLBCL was the most common type of lymphoma in patients diagnosed with HM after SLE; these patients presented with advanced-stage disease and had poor outcomes. In contrast, patients diagnosed with HM before or concurrent with SLE had early stage disease and typically achieved remission.

摘要

目的

红斑狼疮(SLE)患者发生血液系统恶性肿瘤(HMs)的风险高于普通人群。大多数报道都集中在 SLE 后诊断出的 HM 上,并且排除了同时和之前的诊断。关于治疗反应的信息也很有限。

方法

我们在中心确定了 13296 例 HM 和 10539 例潜在 SLE 患者;45 例患者被确诊为 HM 和 SLE。我们的回顾性病例系列基于这 45 例患者。

结果

在这 45 例患者中,64%的患者在诊断为 SLE 后 1 年以上被诊断为 HM,36%的患者在诊断为 SLE 之前或同时患有 HM。在 29 例 SLE 后的 HM 患者中,13 例为弥漫性大 B 细胞淋巴瘤(DLBCL),6 例为惰性淋巴瘤,4 例为白血病,3 例为霍奇金淋巴瘤,1 例为 Burkitt 淋巴瘤、T 细胞淋巴瘤和多发性骨髓瘤。11 例 DLBCL 患者接受环磷酰胺、阿霉素、长春新碱和泼尼松(CHOP)或利妥昔单抗-CHOP 治疗;阿霉素、长春新碱和泼尼松;仅 4 例获得持久缓解。在 16 例 SLE 之前或同时诊断为 HM 的患者中,9 例在 SLE 之前 2 年以上被诊断为 HM,且在 SLE 诊断之前倾向于缓解。7 例患者同时诊断为 HM 和 SLE;就其 HM 而言,6 例达到缓解或稳定疾病。

结论

总之,在 SLE 后诊断出的 HM 患者中,DLBCL 是最常见的淋巴瘤类型;这些患者表现为晚期疾病,预后较差。相比之下,在 SLE 之前或同时诊断出 HM 的患者患有早期疾病,通常会达到缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf24/4246917/0266f686d133/lupus2014000051f01.jpg

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