Hanaoka Mio, Tsukimori Kiyomi, Hojo Satoshi, Abe Yasunobu, Mutou Toshitaka, Muta Koichiro, Iwasa Atsuko, Yao Takashi, Nakano Hitoo
Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Clin Lymphoma Myeloma. 2007 Jul;7(7):486-90. doi: 10.3816/clm.2007.n.033.
We report a case of B-cell lymphoma during pregnancy associated with hemophagocytic syndrome and placental involvement. A 33-year-old Japanese woman developed pancytopenia, hepatosplenomegaly, 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 hemophagocytic syndrome. The screening for infection and autoimmune disease was negative. Clinical manifestation suggested malignant lymphoma as the underlying cause of hemophagocytic syndrome, but we could not confirm any lymphoma involvement in the bone marrow aspiration. Glucocorticoid therapy did not arrest the hemophagocytic 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 immunohistochemical 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 hemophagocytic syndrome during pregnancy, it is important to verify placental microscopic examination for evaluating the causative disease of hemophagocytic syndrome.
我们报告一例妊娠期间发生的B细胞淋巴瘤，伴有噬血细胞综合征和胎盘受累。一名33岁的日本女性在妊娠23周时出现全血细胞减少、肝脾肿大和高热2周。骨髓中噬血细胞的发现证实了噬血细胞综合征的诊断。她在妊娠25周时因噬血细胞综合征转诊进行评估。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是噬血细胞综合征的潜在病因，但骨髓穿刺未证实有任何淋巴瘤累及。糖皮质激素治疗未能阻止噬血细胞过程。她的一般状况恶化，羊水减少。妊娠28周时，因胎儿窘迫行剖宫产。胎盘标本显微镜检查显示绒毛间隙有大量非典型淋巴细胞弥漫浸润。通过免疫组化研究，我们诊断为B细胞淋巴瘤。产后第8天给予R-CHOP（利妥昔单抗/环磷酰胺/阿霉素/长春新碱/泼尼松）化疗。2个周期的R-CHOP化疗后，造血恢复正常，肝脾肿大几乎完全消失。6个周期的R-CHOP化疗后，患者接受了自体外周血干细胞移植，目前诊断后1年处于完全缓解状态。婴儿情况良好，无恶性淋巴瘤的临床或实验室表现。对于妊娠期间怀疑与噬血细胞综合征相关的恶性肿瘤病例，通过胎盘显微镜检查来评估噬血细胞综合征的病因很重要。
