AB039. Complex management of thymic epithelial tumors associated with immuno-haematological disorders at the diagnosis: case reports of our experience

AB039. 诊断时合并免疫血液系统疾病的胸腺上皮肿瘤的复杂管理:我们的病例报告

阅读:2

Abstract

BACKGROUND: Thymic epithelial tumors (TETs) can be associated with immune-haematological disorders that influence the therapeutic approach. The pathogenesis is not well explained and single case reports and small retrospective cohort studies are reported in literature. We present peculiar histories of two patients diagnosed with concurrent subtype A thymoma and immune-haematological disorders at diagnosis. CASE DESCRIPTION: The first patient, 68 years old, in March 2023 underwent surgery for anterior mediastinal mass. Diagnosis of stage I subtype A thymoma was made. Subsequently, Good’s syndrome was diagnosed. In April 2023, the patient was affected by SARS-Cov-2 infection and treatment with antiviral drugs was promptly administered, resulting in a resolution of symptoms, but tests for SARS-COVID-2 viremia were still positive. The patient presented several life-threating upper respiratory tract infectious diseases by various pathogen agents and was continuously hospitalized. In April 2024, for anaemia and thrombocytopenia, osteo-medullary biopsy (BOM) was performed. Medullary aplasia was diagnosed, then corticosteroids, cyclosporine, immunoglobulins and blood transfusion were promptly administered. In May 2024, the radiological evaluation for TET was negative. The second patient, 60 years old, underwent biopsy of a mass in anterior mediastinum in July 2023, showing a subtype A thymoma. Subsequently, for the onset of grade 3 anaemia and grade 4 thrombocytopenia, BOM was performed revealing medullary aplasia. Anaemia and thrombocytopenia prevented curative surgery or systemic treatment for TET. Treatment with corticosteroids, cyclosporine, blood transfusions and antilymphocyte serum was ineffective and the patient died in March 2024. The cases we present support the evidence that thymoma A can be insidious for association with immune-haematological diseases that can preclude the treatment of TET, although is considered to be biologically less aggressive histotype. We review literature finding that immune-haematological disorders are less than 1% of cases of TETs and regroup several clinical conditions, including pure red cell aplasia (occurring in 5% of thymomas), aplastic anaemia, haemolytic anaemia, paroxysmal nocturnal haemoglobinuria, pure white cell aplasia and acquired amegakaryocytic thrombocytopenia. CONCLUSIONS: The history of these two patients show that the management of patients with TETs and medullary disorders at diagnosis condition the therapeutic process of thymic tumors, influencing prognosis.

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。