title: B-cell lymphoma during pregnancy associated with hemophagocytic syndrome and placental involvement.
abstract: 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.
authors: Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors: Mio
authors: M
authors: Hanaoka
authors: Kiyomi
authors: K
authors: Tsukimori
authors: Satoshi
authors: S
authors: Hojo
authors: Yasunobu
authors: Y
authors: Abe
authors: Toshitaka
authors: T
authors: Mutou
authors: Koichiro
authors: K
authors: Muta
authors: Atsuko
authors: A
authors: Iwasa
authors: Takashi
authors: T
authors: Yao
authors: Hitoo
authors: H
authors: Nakano
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002585:Cesarean Section; D005260:Female; D005316:Fetal Distress / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D005865:Gestational Age; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D036102:Peripheral Blood Stem Cell Transplantation; D010920:Placenta / Q000473:pathology; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D011252:Pregnancy Complications, Neoplastic / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D014182:Transplantation, Autologous