Autoimmune hemolytic anemia and thrombocytopenia attributed to an intrauterine contraceptive device. Transfusion 2015 Mar;55(3):657-60
Date
09/12/2014Pubmed ID
25208591Pubmed Central ID
PMC4372189DOI
10.1111/trf.12865Scopus ID
2-s2.0-84924430382 (requires institutional sign-in at Scopus site) 2 CitationsAbstract
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.
CASE REPORT: 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.
CONCLUSION: 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.
Author List
Khawandanah MO, Weiss SM, Cherry MA, Maymani H, Selby GB, Aster RH, George JN, Holter Chakrabarty JLMESH terms used to index this publication - Major topics in bold
AdultAgglutination Tests
Alemtuzumab
Anemia, Hemolytic, Autoimmune
Animals
Antibodies, Monoclonal, Humanized
Blood Transfusion
Combined Modality Therapy
Device Removal
Drug Resistance
Female
Humans
Immunosuppressive Agents
Intrauterine Devices, Medicated
Levonorgestrel
Mice
Mice, Inbred NOD
Mice, SCID
Plasma Exchange
Polyethylene
Polyethylene Glycols
Splenectomy
Thrombocytopenia