title: Autoimmune hemolytic anemia and thrombocytopenia attributed to an intrauterine contraceptive device.
abstract: BACKGROUND
  Evans syndrome is a rare condition manifested by combined autoimmune hemolytic anemia (AIHA) and thrombocytopenia or neutropenia. It is often associated with other autoimmune disorders, immunodeficiencies, and non-Hodgkin's lymphoma.
  
  
  METHODS
  We describe a patient with Evans syndrome that may have been related to exposure to a polyethylene-based intrauterine contraceptive device (IUD). A 26-year-old white female presented with severe, symptomatic AIHA and subsequently developed severe thrombocytopenia. She had a refractory course resistant to multiple treatments including corticosteroids, intravenous immune globulin, rituximab, splenectomy, cyclophosphamide, cyclosporine, eculizumab, and plasma exchange. It was then noticed that her serum autoantibody agglutinated red blood cells (RBCs) in the presence of polyethylene glycol (PEG) but not in the absence of PEG nor when an alternative agglutination enhancing technique, low-ionic-strength solution, was used. Therefore, her polyethylene-containing IUD, which was a polyethylene frame with a levonorgestrel-releasing device, was removed. Norgestrel-dependent, platelet (PLT)-reactive antibodies were not identified by either flow cytometry or in vivo in a NOD/SCID mouse. Testing for PEG-dependent antibodies was not possible. Remission, with no requirement for RBC or PLT transfusions and return of her hemoglobin and PLT counts to normal, followed removal of the IUD.
  
  
  CONCLUSIONS
  The patient's recovery after removal of the IUD and the PEG dependence of RBC agglutination suggested a possibility that the IUD may have been a contributing factor to the etiology of Evans syndrome in this patient.
authors.affiliation: Hematology-Oncology Section, Department of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma.
authors.forename: Mohamad O
authors.initials: MO
authors.lastname: Khawandanah
authors.forename: Susan M
authors.initials: SM
authors.lastname: Weiss
authors.forename: Mohamad A
authors.initials: MA
authors.lastname: Cherry
authors.forename: Hossein
authors.initials: H
authors.lastname: Maymani
authors.forename: George B
authors.initials: GB
authors.lastname: Selby
authors.forename: Richard H
authors.initials: RH
authors.lastname: Aster
authors.forename: James N
authors.initials: JN
authors.lastname: George
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Holter Chakrabarty
mesh_terms: D000328:Adult; D000372:Agglutination Tests; D000074323:Alemtuzumab; D000744:Anemia, Hemolytic, Autoimmune / Q000139:chemically induced* / Q000188:drug therapy / Q000276:immunology / Q000628:therapy; D000818:Animals; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001803:Blood Transfusion; D003131:Combined Modality Therapy; D020878:Device Removal; D004351:Drug Resistance; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007436:Intrauterine Devices, Medicated / Q000009:adverse effects*; D016912:Levonorgestrel; D051379:Mice; D016688:Mice, Inbred NOD; D016513:Mice, SCID; D010951:Plasma Exchange; D020959:Polyethylene / Q000009:adverse effects*; D011092:Polyethylene Glycols / Q000009:adverse effects*; D013156:Splenectomy; D013921:Thrombocytopenia / Q000139:chemically induced* / Q000188:drug therapy / Q000276:immunology / Q000628:therapy
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; D000074323:Alemtuzumab; D011092:Polyethylene Glycols; D016912:Levonorgestrel; D020959:Polyethylene; C481642:eculizumab