Progressive multifocal leukoencephalopathy associated with thymoma with immunodeficiency: a case report and literature review.
Progressive multifocal leukoencephalopathy associated with thymoma with immunodeficiency: a case report and literature review.
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DOI：
10.1186/s12883-018-1041-4
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发表时间：
2018-04-10
期刊：
影响因子：
2.6
通讯作者：
Tomiyama M
中科院分区：
文献类型：
--
作者：
Ueno T;Sato N;Kon T;Haga R;Nunomura JI;Nakamichi K;Saijo M;Tomiyama M
进行性多灶性脑白质病（PML）的发展与严重的细胞免疫抑制有关。Good’s syndrome （GS）是一种罕见的胸腺瘤相关免疫缺陷综合征，以体液和细胞免疫抑制为主；然而，由于GS引起的PML的报道很少。一份报告显示，减少重症肌无力（MG）的免疫抑制治疗，可能会改善与胸腺瘤相关的PML神经系统症状。因此，有必要确定PML患者免疫缺陷的原因，以便采取适当的治疗策略。一名47岁的日本女性因失语和步态困难入院。她患有侵袭性胸腺瘤，曾多次接受化疗，包括环磷酰胺。她之前也被诊断为MG（美国重症肌无力基金会临床分类IIa），但她的上睑下垂和四肢无力已经完全恢复。入院时，神经学检查显示运动失语症和右侧中央面部无力。实验室研究显示严重淋巴细胞减少，CD4+和CD8+ T细胞和CD19+ B细胞计数减少，所有免疫球蛋白亚类水平降低，提示GS。人类免疫缺陷病毒（HIV）血清学检测结果为阴性。脑磁共振成像显示非对称多灶性白质病变，无增强。脑脊液实时聚合酶链反应阳性，检测结果为628.3万份/mL。我们诊断为非hiv相关PML合并GS和可能的化疗诱导免疫缺陷。随后，她接受了静脉注射免疫球蛋白、米氮平和甲氟喹治疗，但在入院后46天死于败血症。在胸腺瘤相关的PML患者中，有必要考虑GS导致免疫缺陷的可能性。神经科医生应牢记MG患者合并胸腺瘤发生PML的风险，即使MG症状缓解，也应据此评估患者的免疫状况。
The development of progressive multifocal leukoencephalopathy (PML) is associated with severe cellular immunosuppression. Good’s syndrome (GS) is a rare immunodeficiency syndrome related to thymoma, with the development of humoral as well as cellular immunosuppression; however, there are few reports of PML due to GS. One report suggested that the neurological symptoms of PML related to thymoma may be improved by a reduction of immunosuppressive therapy for myasthenia gravis (MG). It is therefore necessary to identify the cause of immunodeficiency in patients with PML to enable an appropriate treatment strategy to be adopted. A 47-year-old Japanese woman was admitted with aphasia and gait difficulty. She had an invasive thymoma that had been treated with repeated chemotherapy, including cyclophosphamide. She had also previously been diagnosed with MG (Myasthenia Gravis Foundation of America clinical classification IIa), but her ptosis and limb weakness had completely recovered. On admission, neurological examination revealed motor aphasia and central facial weakness on the right side. Laboratory studies showed severe lymphopenia, decreased CD4+ and CD8+ T cell and CD19+ B cell counts, and reduced levels of all subclasses of immunoglobulins, suggesting GS. Serology for human immunodeficiency virus (HIV) infection was negative. Brain magnetic resonance imaging showed asymmetric multifocal white matter lesions without contrast enhancement. Cerebrospinal fluid real-time polymerase chain reaction for JC virus was positive, showing 6,283,000 copies/mL. We made a diagnosis of non-HIV-related PML complicated with GS and probable chemotherapy-induced immunodeficiency. She then received intravenous immunoglobulin therapy, mirtazapine, and mefloquine, but died of sepsis 46 days after admission. It is necessary to consider the possibility of immunodeficiency due to GS in patients with PML related to thymoma. Neurologists should keep in mind the risk of PML in MG patients with thymoma, even if the MG symptoms are in remission, and should thus evaluate the immunological status of the patient accordingly.
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DOI：
10.1016/j.clim.2010.01.006
发表时间：
2010-06
期刊：
Clinical immunology (Orlando, Fla.)
影响因子：
--
作者：
Kelesidis T;Yang O
通讯作者：
Yang O
影响因子：
2.8
作者：
Gedizhoglu, Muhtesem;Coban, Pinar;Sivasli, Izlem Evren
通讯作者：
Sivasli, Izlem Evren
影响因子：
45.3
作者：
Heine, Annkristin;Schmiedel, Alexandra;Brossart, Peter
通讯作者：
Brossart, Peter
影响因子：
0.9
作者：
Sveinsson, Olafur;Matell, Henrik;Herrman, Lars
通讯作者：
Herrman, Lars
影响因子：
158.5
作者：
MACKALL, CL;FLEISHER, TA;GRESS, RE
通讯作者：
GRESS, RE