Autoimmune hemolytic anemia and thrombocytopenia attributed to an intrauterine contraceptive device. Issue 3 (11th September 2014)

• Record Type: Journal Article
• Authors: Khawandanah, Mohamad O.; Weiss, Susan M.; Cherry, Mohamad A.; Maymani, Hossein; Selby, George B.; Aster, Richard H.; George, James N.; Holter Chakrabarty, Jennifer L.
• Is Part Of: Transfusion. Volume 55:Issue 3(2015)
• Page Start: 657
• Page End: 660
• Publication Date: 2014-09-11
• Subjects: Hematology -- Periodicals; Blood -- Transfusion -- Periodicals; Blood Group Antigens -- Periodicals; Blood Preservation -- Periodicals; Blood Transfusion -- Periodicals; 615
• DOI: 10.1111/trf.12865
• Languages: English
• ISSNs: 0041-1132

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="trf12865-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="trf12865-sec-0002" sec-type="section"> <title>Case Report</title> <p>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.</p> </sec> <sec id="trf12865-sec-0003" sec-type="section"> <title>Conclusion</title> <p>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.</p> </sec> </abstract>