Primary Sjögren Syndrome Presenting with Hemolytic Anemia and Pure Red Cell Aplasia Following Delivery due to Coombs-negative Autoimmune Hemolytic Anemia and Hemophagocytosis

Yohei Komaru; Takakazu Higuchi; Ryosuke Koyamada; Youichiro Haji; Masato Okada; Toyomi Kamesaki; Sadamu Okada

doi:10.2169/internalmedicine.52.0695

CASE REPORTS

Primary Sjögren Syndrome Presenting with Hemolytic Anemia and Pure Red Cell Aplasia Following Delivery due to Coombs-negative Autoimmune Hemolytic Anemia and Hemophagocytosis

Yohei Komaru, Takakazu Higuchi, Ryosuke Koyamada, Youichiro Haji, Masato Okada, Toyomi Kamesaki, Sadamu Okada

Author information

Keywords: Sjögren syndrome, autoimmune hemolytic anemia, hemophagocytosis, pure red cell aplasia, pregnancy, delivery

JOURNAL OPEN ACCESS

2013 Volume 52 Issue 20 Pages 2343-2346

DOI https://doi.org/10.2169/internalmedicine.52.0695

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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.

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