title: Primary Sjögren syndrome presenting with hemolytic anemia and pure red cell aplasia following delivery due to Coombs-negative autoimmune hemolytic anemia and hemophagocytosis.
abstract: A 36-year-old woman presented with hemolytic anemia without a reticulocyte response 38 days after delivery. A marked reduction in erythroid cells and an increase in macrophages with active hemophagocytosis were noted in the bone marrow. While conventional Coombs' tests were negative, the level of red blood cell (RBC)-bound immunoglobulin G (IgG) was increased. The patient was diagnosed with primary Sjögren syndrome (pSS) based on her symptoms, positive anti-SS-A antibodies, Coombs-negative autoimmune hemolytic anemia and pure red cell aplasia associated with RBC-bound IgG and hemophagocytosis. The unique presentation was considered to be a consequence of immunological derangement associated with pSS, pregnancy and delivery.
authors.affiliation: Division of Hematology, St. Luke's International Hospital, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Komaru
authors.forename: Takakazu
authors.initials: T
authors.lastname: Higuchi
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Koyamada
authors.forename: Youichiro
authors.initials: Y
authors.lastname: Haji
authors.forename: Masato
authors.initials: M
authors.lastname: Okada
authors.forename: Toyomi
authors.initials: T
authors.lastname: Kamesaki
authors.forename: Sadamu
authors.initials: S
authors.lastname: Okada
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000175:diagnosis* / Q000209:etiology; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000175:diagnosis*; D003298:Coombs Test / Q000379:methods; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000175:diagnosis*; D011247:Pregnancy; D011644:Puerperal Disorders / Q000175:diagnosis* / Q000209:etiology; D012010:Red-Cell Aplasia, Pure / Q000178:diet therapy* / Q000209:etiology; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000209:etiology