Splenectomy Linking Guillain-Barré Syndrome, Autoimmune Haemolysis, and Epstein-Barr Virus Viraemia: Unveiling a Hidden Diagnosis of Angioimmunoblastic T-cell Lymphoma

脾切除术揭示格林-巴利综合征、自身免疫性溶血和EB病毒血症之间的联系:揭示血管免疫母细胞性T细胞淋巴瘤的隐匿诊断

阅读:1

Abstract

A 64-year-old man presented to the emergency department of a district general hospital with a widespread rash. He received antihistamine treatment and was discharged. He subsequently returned with peripheral paraesthesia and severe back pain. Neurological assessment showed pronounced bilateral ascending lower limb motor weakness, and further review found diplopia and dysphagia. A diagnosis of Guillain-Barré syndrome (GBS) with the Miller Fisher variant was established, and treatment with high-dose intravenous immunoglobulin (IVIG) was initiated. The patient was transferred to a tertiary centre for nerve conduction studies. During repatriation, the patient developed severe hyponatraemia, and paired serum and urine osmolality testing indicated syndrome of inappropriate antidiuretic hormone secretion (SIADH). A staging CT discovered numerous borderline enlarged lymph nodes, seemingly consistent with a viral screen, which identified an Epstein-Barr virus (EBV) viraemia. A rapid drop in the patient's haemoglobin alongside a positive direct antibody test prompted a further diagnosis of autoimmune haemolytic anaemia (AIHA). High-dose steroids and rituximab both failed to abate the haemolysis, necessitating an ITU admission for stabilisation and emergency splenectomy. Histopathological analysis of the spleen was consistent with angioimmunoblastic T-cell lymphoma (AITL), providing a unifying explanation for the patient's diverse autoimmune manifestations. The patient was initiated on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy, and he achieved a complete metabolic response on interim PET-CT, and remains in remission at nine months of follow-up. This presentation of AITL with concurrent GBS and AIHA highlights the potential for this disease to present primarily with features of immune dysregulation.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。