B-cell lymphoma during pregnancy associated with hemophagocytic syndrome and placental involvement
B-cell lymphoma during pregnancy associated with hemophagocytic syndrome and placental involvement
复制标题
DOI：
10.3816/clm.2007.n.033
复制
发表时间：
2007-07-01
期刊：
影响因子：
--
通讯作者：
Nakano, Hitoo
中科院分区：
文献类型：
--
作者：
Hanaoka, Mio;Tsukimori, Kiyomi;Nakano, Hitoo
我们报告一个怀孕期间发生B细胞淋巴瘤的病例，合并有噬疝细胞症候群及胎盘受累。一名33岁的日本女性在妊娠23周时出现全血细胞减少、肝脾阴性和高热2周。她骨髓中的噬血细胞证实了噬血细胞综合征的诊断。她在妊娠25周时转诊，以评价噬疝细胞综合征。感染和自身免疫性疾病筛查均为阴性。临床表现提示恶性淋巴瘤为巨噬细胞综合征的潜在病因，但我们无法在骨髓穿刺中证实任何淋巴瘤受累。糖皮质激素治疗没有阻止hernophagocytic过程。她的一般状况恶化，并注意到羊水减少。在妊娠28周时，我们因胎儿窘迫进行了剖腹产。胎盘标本的显微镜检查显示大的非典型淋巴细胞弥漫性浸润，累及绒毛间隙。经病理学检查，诊断为B细胞淋巴瘤。R-CHOP（利妥昔单抗/环磷酰胺/多柔比星/长春新碱/泼尼松）化疗在产后第8天进行。R-CHOP方案化疗2周期后，造血功能恢复正常，肝脾肿大基本消失。经过6个周期的R-CHOP治疗后，患者接受了自体外周血干细胞移植，目前诊断后1年完全缓解。婴儿情况良好，无恶性淋巴瘤的临床或实验室表现。对于妊娠期疑似恶性肿瘤伴噬血细胞综合征的病例，重要的是要验证胎盘显微镜检查，以评估噬血细胞综合征的病因。
We report a case of B-cell lymphoma during pregnancy associated with hernophagocytic syndrome and placental involvement. A 33-year-old Japanese woman developed pancytopenia, hepatosplenornegaly, and a high-grade fever for 2 weeks at 23 weeks of gestation. The demonstration of hemophagocytes in her bone marrow confirmed the diagnosis of hemophagocytic syndrome. She was referred at 25 weeks of gestation for evaluation of hernophagocytic syndrome. The screening for infection and autoimmune disease was negative. Clinical manifestation suggested malignant lymphoma as the underlying cause of hernophagocytic syndrome, but we could not confirm any lymphoma involvement in the bone marrow aspiration. Glucocorticoid therapy did not arrest the hernophagocytic process. Her general status worsened, and reduction of amniotic fluid was noted. At 28 weeks of gestation, we performed a Cesarean section because of fetal distress. Microscopic examination of placental specimen revealed diffuse infiltration of large, atypical lymphoid cells involving the intervillous space. Using immunohistochernical study, we made the diagnosis of B-cell lymphoma. R-CHOP (rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy was administered on the eighth postpartum day. After 2 cycles of R-CHOP chemotherapy, hematopoiesis became normal and hepatosplenomegaly almost completely disappeared. After 6 cycles of R-CHOP, the patient received autologous peripheral-blood stem cell transplantation, and she is currently in complete remission 1 year after diagnosis. The infant did well, without clinical or laboratory manifestations of malignant lymphoma. In cases with suspected malignancy associated with hernophagocytic syndrome during pregnancy, it is important to verify placental microscopic examination for evaluating the causative disease of hemophagocytic syndrome.