Successful Artery Embolization in a Patient with Autoimmune Lymphoproliferative Syndrome Associated with Splenic Rupture
Successful Artery Embolization in a Patient with Autoimmune Lymphoproliferative Syndrome Associated with Splenic Rupture
复制标题
与脾破裂相关的自身免疫性淋巴增殖综合征患者的成功动脉栓塞
DOI：
10.1007/s10875-020-00809-3
复制
发表时间：
2020
影响因子：
9.1
通讯作者：
Kensaku Kohrogi et al.
中科院分区：
文献类型：
--
作者：
Kato Yu;Kunimasa Kazuhiro;Takahashi Mizuki;Harada Ayaka;Nagasawa Ikuko;Osawa Masanori;Sugimoto Yoshikazu;Tomida Akihiro;Go J. Yoshida;村上翔子;Kensaku Kohrogi et al.
编辑认为，自身免疫性淋巴组织增生综合征（ALPS）是一种淋巴细胞稳态的常染色体显性遗传疾病，患者表现为脾肿大和淋巴结病[1]。ALPS由T淋巴细胞的缺陷性凋亡和T细胞受体α/β链阳性和CD 4和CD 8阴性的双阴性T（α/β-DNT）细胞的积累引起。这导致淋巴结病和脾肿大在生命的早期。ALPS患者的外周血和淋巴组织中α/β-DNT细胞水平升高[1]。ALPS的治疗包括类固醇和免疫抑制剂，以改善免疫性血细胞减少症。在最近的一项前瞻性多机构试验中，西罗莫司被证明对自身免疫性血细胞减少症有效[2]。脾肿大是ALPS的并发症之一，脾肿大明显的患者被迫限制日常活动以防止脾破裂。ALPS患者应避免脾切除术，因为其对免疫性血细胞减少症的有效性有限，且严重感染的风险较高[3]。然而，目前尚无治疗ALPS患者脾破裂的指南。小儿脾破裂是一种危及生命的器官损伤，在某些情况下需要立即干预。虽然脾破裂的主要原因是强烈的钝性创伤，但在脾肿大患者中也曾报告过特发性脾破裂或轻微创伤后脾破裂[4]。在这种情况下，了解根本原因对于设计适当的治疗计划至关重要。然而，脾肿大的确切原因并不总是确定的。
To the Editor, Autoimmune lymphoproliferative syndrome (ALPS) is an autosomal dominant disorder of lymphocyte homeostasis wherein patients present with splenomegaly and lymphadenopathy [1]. ALPS results from defective apoptosis of T lymphocytes and accumulation of the T cell receptor α/β chainpositive and CD4 and CD8-negative double negative T (α/β-DNT) cells. This leads to lymphadenopathy and splenomegaly during the early years of life. Patients with ALPS exhibit elevated levels of α/β-DNT cells in the peripheral blood and lymphoid tissues [1]. Therapy for ALPS comprises steroids and immunosuppressants to improve immune cytopenia. Sirolimus was shown to be effective in autoimmune cytopenia in a recent prospective multi-institutional trial [2]. Splenomegaly is one of the complications of ALPS, and patients with remarkable splenomegaly are forced to limit their daily activities to prevent splenic rupture. Splenectomy should be avoided in ALPS patients owing to its limited efficiency for immune cytopenia and associated high risk of severe infection [3]. However, there are no treatment guidelines for managing splenic rupture in ALPS patients. Pediatric splenic rupture is a life-threatening organ injury that requires immediate intervention in certain cases. Although the main cause of splenic rupture is strong blunt trauma, idiopathic splenic rupture or splenic rupture following minor trauma has been reported in patients with splenomegaly [4]. In such cases, understanding the underlying cause (s) is imperative to design an appropriate treatment plan. However, the exact cause of splenomegaly cannot always be determined.
登录
查看更多内容
影响因子：
20.3
作者：
Bride, Karen L.;Vincent, Tiffaney;Teachey, David T.
通讯作者：
Teachey, David T.
疑似腹部钝性损伤患者腹部 CT 阴性后无需入院或观察：一项前瞻性、多机构试验的结果
DOI：
--
发表时间：
1997
期刊：
影响因子：
--
作者：
D. Livingston;R. Lavery;M. Passannante;T. Fabian;D. Fry;Malangoni
通讯作者：
Malangoni
采用氯法拉滨/白消安 × 4 (CloBu4) 调理的同种异体造血干细胞移植在非缓解性血液恶性肿瘤（尤其是 AML）中表现出显着的抗肿瘤活性
影响因子：
20.3
作者：
S. Mineishi;J. Magenau;H. Tobai;A. Pawarode;E. Peres;P. Reddy;C. Kitko;S. Choi;H. Erba;Lisa A Kujawski;G. Yanik;J. Ferrara;J. Levine
通讯作者：
J. Levine
影响因子：
20.3
作者：
Neven, Benedicte;Magerus-Chatinet, Aude;Rieux-Laucat, Frederic
通讯作者：
Rieux-Laucat, Frederic
35 名癌症患者的部分脾动脉栓塞：单一机构回顾性研究的结果。
DOI：
10.1016/j.jvir.2019.05.031
发表时间：
2020
期刊：
Journal of vascular and interventional radiology : JVIR
影响因子：
--
作者：
B. Kis;M. Mills;Johnna Smith;Junsung Choi;Elizabeth M. Sagatys;R. Komrokji;J. Strosberg;R. Kim
通讯作者：
R. Kim