Kittan N A, Beier F, Kurz K, Niller H H, Egger L, Jilg W, Andreesen R, Holler E, Hildebrandt G C
Department of Hematology and Oncology, University of Regensburg Medical Center, Regensburg, Germany.
Transpl Infect Dis. 2011 Oct;13(5):524-30. doi: 10.1111/j.1399-3062.2011.00621.x. Epub 2011 Mar 13.
We present the case of a 49-year-old male patient with Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) limited to the brain that occurred 6 months after allogeneic hematopoietic stem cell transplantation (HSCT). Clinical symptoms included mental confusion, ataxia, and diplopia. Magnetic resonance imaging (MRI) revealed cerebellar and periventricular lesions consistent with an inflammatory process. Cerebrospinal fluid (CSF) analysis, but not peripheral blood, was positive for EBV-DNA, but no malignant cells were found. Brain biopsy was not feasible because of low platelet counts. As we considered a diagnosis of either EBV-associated encephalitis or PTLD, the patient was treated with rituximab combined with antiviral therapy. However, the cerebral lesions progressed and follow-up CSF testing revealed immunoglobulin H clonality as evidence of a malignant process. Subsequent treatment attempts included 2 donor lymphocyte infusions (DLI). Despite treatment, the patient died from autopsy-proven PTLD within 8 weeks of the onset of symptoms. This case demonstrates the clinical and diagnostic challenges of primary cerebral PTLD in a patient following allogeneic HSCT.
我们报告了一例49岁男性患者，其在异基因造血干细胞移植（HSCT）后6个月发生了局限于脑部的与爱泼斯坦-巴尔病毒（EBV）相关的移植后淋巴细胞增殖性疾病（PTLD）。临床症状包括精神错乱、共济失调和复视。磁共振成像（MRI）显示小脑和脑室周围病变，与炎症过程相符。脑脊液（CSF）分析显示EBV-DNA呈阳性，但外周血检测为阴性，且未发现恶性细胞。由于血小板计数低，脑活检不可行。鉴于我们考虑诊断为EBV相关脑炎或PTLD，该患者接受了利妥昔单抗联合抗病毒治疗。然而，脑部病变仍进展，后续脑脊液检测显示免疫球蛋白H克隆性，提示存在恶性过程。随后的治疗尝试包括2次供体淋巴细胞输注（DLI）。尽管进行了治疗，患者在症状出现后8周内死于经尸检证实的PTLD。本病例展示了异基因HSCT患者原发性脑PTLD的临床和诊断挑战。
