Division of Obstetrics, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-Ku, Tokyo, Japan.
Division of Maternal Medicine, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-Ku, Tokyo, Japan.
BMC Pregnancy Childbirth. 2022 Oct 27;22(1):795. doi: 10.1186/s12884-022-05145-5.
Pure red cell aplasia (PRCA) is a hematological disorder characterized by anemia with severe reticulocytopenia caused by a marked reduction in erythroid precursors in the bone marrow. PRCA is known to be associated with pregnancy, but thymoma-associated PRCA during pregnancy is very rare, and its successful management has not been reported.
A 37-year-old primiparous woman with severe anemia was referred to our center at 27 weeks' gestation. She was diagnosed with PRCA based on bone aspiration findings at 33 weeks' gestation. Magnetic resonance imaging (MRI) revealed an anterior mediastinal mass 4 cm in size suspected of being thymoma. She was therefore diagnosed with thymoma-associated PRCA during pregnancy. Surgery for thymoma was planned after delivery, since the imaging findings were suggestive of early-stage thymoma (Masaoka stage I or II). With transfusion of a total 3,360 ml of red blood cells (RBCs) during pregnancy, the patient gave birth to a baby girl weighing 2,548 g at 40 weeks' gestation. The baby showed transient congenital cutaneous candidiasis. The placental pathology revealed subamniotic inflammation with a fungal structure. Treatment with topical anti-fungal cream immediately ameliorated the baby's skin lesion. Maternal anemia did not improve after delivery; however, the thymoma did not increase in size. At five months after delivery, the mother underwent thymectomy with oral cyclosporine A. A pathological examination revealed Masaoka stage II-a thymoma. She completely had recovered from anemia at six months after surgery. Cyclosporine A treatment was discontinued three years after surgery. Remission has been sustained for four years since surgery.
A very rare case of thymoma-associated PRCA during pregnancy was diagnosed without any subjective symptoms and was expectantly managed, resulting in a good prognosis. Although bone marrow aspiration during pregnancy is an invasive test, it is important to confirm the diagnosis. Conservative management with blood transfusion was possible for early-stage thymoma-associated PRCA during pregnancy. Active surveys, including MRI, for PRCA during pregnancy led to the detection of thymoma at an early stage and the achievement of a preferable pregnancy outcome.
纯红细胞再生障碍性贫血（PRCA）是一种血液学疾病，其特征为贫血伴有网织红细胞严重减少，这是由于骨髓中红系前体细胞明显减少所致。已知 PRCA 与妊娠有关，但妊娠期间伴发胸腺瘤的 PRCA 非常罕见，且其成功管理尚未有报道。
一位 37 岁初产妇，妊娠 27 周时因严重贫血被转诊至我院。妊娠 33 周时，根据骨髓抽吸结果诊断为 PRCA。磁共振成像（MRI）显示前纵隔 4cm 大小的肿块，疑似胸腺瘤。因此，该患者被诊断为妊娠期间伴发胸腺瘤的 PRCA。由于影像学检查提示为早期胸腺瘤（Masaoka Ⅰ期或Ⅱ期），计划在分娩后进行胸腺瘤手术。在妊娠期间共输注了 3360ml 的红细胞（RBC）后，该患者于妊娠 40 周时分娩出一名体重为 2548g 的女婴。该婴儿出现短暂性先天性皮肤念珠菌病。胎盘病理学显示亚羊膜炎症伴真菌结构。立即给予局部抗真菌乳膏治疗，婴儿的皮肤病变得到改善。分娩后，产妇的贫血并未改善；然而，胸腺瘤并未增大。分娩后 5 个月，该患者接受了胸腺切除术联合口服环孢素 A。病理检查显示 Masaoka Ⅱ-a 期胸腺瘤。术后 6 个月，她完全摆脱了贫血。术后 3 年停用环孢素 A。自手术后 4 年，疾病一直处于缓解状态。
本病例为妊娠期间伴发胸腺瘤的非常罕见的 PRCA，患者无任何主观症状，经期待治疗后获得了良好的预后。尽管妊娠期间骨髓抽吸是一种有创性检查，但对于明确诊断非常重要。对于妊娠期间早期伴发胸腺瘤的 PRCA，可通过输血进行保守治疗。对 PRCA 进行积极的包括 MRI 在内的检查，可在早期发现胸腺瘤，从而获得更好的妊娠结局。
