Abdulgayoom Mohammed, Ganwo Ibrahim, Ata Fateen, Surchi Haval, Al-Homsi Ussama, Cherif Honar
Department of Hematology, National Center for Cancer Care and Research, HMC, Doha, Qatar.
Department of Hematopathology, National Center for Cancer Care and Research, HMC, Doha, Qatar.
Case Rep Oncol. 2025 Jan 14;18(1):100-106. doi: 10.1159/000542915. eCollection 2025 Jan-Dec.
Pure red cell aplasia (PRCA) is a rare cause of anemia characterized by decreased red blood cell production, often associated with thymomas. While thymectomy is commonly performed to treat thymoma-associated PRCA, the development of PRCA post-thymectomy is less understood.
We present a case of a 25-year-old woman who developed PRCA and autoimmune diabetes mellitus (DM) following radical thymectomy for malignant thymoma. Initial treatment with prednisolone showed minimal improvement, leading to the inclusion of rituximab. This combination resulted in a significant increase in reticulocyte counts and transfusion independence.
This case highlights the potential for PRCA to develop after thymectomy and the challenges of treating PRCA in the context of additional conditions like autoimmune DM. While immunosuppressive therapies such as cyclosporin A and corticosteroids are standard, their side effects necessitated exploring alternative treatments. Rituximab proved effective and was well-tolerated, suggesting its potential as a primary option in similar cases.
This case illustrates the occurrence of PRCA after thymectomy and highlights rituximab's effectiveness as a treatment option, offering a viable alternative to standard therapies in managing this complex condition.
纯红细胞再生障碍性贫血（PRCA）是一种罕见的贫血病因，其特征为红细胞生成减少，常与胸腺瘤相关。虽然胸腺切除术常用于治疗胸腺瘤相关的PRCA，但胸腺切除术后PRCA的发生情况尚不太清楚。
我们报告一例25岁女性，她在因恶性胸腺瘤行根治性胸腺切除术后发生了PRCA和自身免疫性糖尿病（DM）。最初使用泼尼松龙治疗改善甚微，随后加用了利妥昔单抗。这种联合治疗使网织红细胞计数显著增加，并实现了输血独立。
该病例突出了胸腺切除术后发生PRCA的可能性，以及在自身免疫性DM等附加疾病背景下治疗PRCA的挑战。虽然环孢素A和皮质类固醇等免疫抑制疗法是标准治疗方法，但它们的副作用使得有必要探索其他治疗方法。利妥昔单抗被证明有效且耐受性良好，表明其在类似病例中作为主要治疗选择的潜力。
本病例说明了胸腺切除术后PRCA的发生情况，并突出了利妥昔单抗作为一种治疗选择的有效性，为管理这种复杂疾病提供了一种可行的标准治疗替代方案。
