• Yang Yang, Chunmei Chen, Bingrong Zheng, Liping Fan, Xiajun Chen, and Meiwei Hu.
• Department of Hematology, the Second Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China.
• Medicine (Baltimore). 2024 Jan 19; 103 (3): e36920e36920.

RationalePure white cell aplasia (PWCA) is a rare paraneoplastic syndrome that occurs in patients with thymomas. Currently, the pathogenesis and treatment of this disease remain in the exploratory stage.Patient ConcernsWe report a 68-year-old woman with thymoma experienced PWCA involvement as her first presentation. The patient had high fever and agranulocytosis at the onset of the disease. The white blood cell count in the complete blood count was 1.9 × 109/L with a neutrophil of 0.1 × 109/L. The bone marrow aspirates showed decreased granulocyte proliferation. Computed tomography showed a large mass in the anterior mediastinum.DiagnosesThe final diagnosis of our patient was PWCA and thymoma.InterventionsShe underwent a thymectomy and cyclosporine A administration during first remission.OutcomesLong-term remission was achieved following the readministration of cyclosporine A after the disease recurrence.LessonsPWCA or agranulocytosis with thymoma has been confirmed to be an extremely rare disease. Thymomas with PWCA correlate with autoimmunity. From this case study and the literature review, we concluded that the pathogenesis of thymomas in PWCA is mainly related to the activation of autoreactive T cells. Thymectomy and the immunosuppressive drug, cyclosporine A, were chosen for treatment. The patient's granulocyte levels were unable to recover after surgery because of the inability to promptly clear activated T cells. After surgery, cyclosporine A continued to take for a long time. Thymectomy combined with prolonged cyclosporine A administration may be an effective method for treating this rare disease.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

31 keywords...

hide…