Hindawi Publishing Corporation Case Reports in Obstetrics and Gynecology Volume 2013, Article ID 413502, 4 pages http://dx.doi.org/10.1155/2013/413502 Case Report Pregnancy with a Severe Hemoglobinopathy: Unintended Consequences of Transfusions David Kim, 1 Hector Mendez-Figueroa, 2 and Brenna L. Anderson 2 1 Department of Obstetrics and Gynecology, Women & Infants Hospital, he Warren Alpert Medical School of Brown University, Providence, RI 02905, USA 2 Maternal-Fetal Medicine Clinic, Department of Obstetrics and Gynecology, Women & Infants Hospital, he Warren Alpert Medical School of Brown University, 101 Dudley Street, 3rd Floor, Providence, RI 02905-2401, USA Correspondence should be addressed to Brenna L. Anderson; banderson@wihri.org Received 9 November 2012; Accepted 10 January 2013 Academic Editors: O. Oyesanya and A. Semczuk Copyright © 2013 David Kim et al. his is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. We report a case of a pregnant woman with a complex hemoglobinopathy who developed a symptomatic anemia at 28 weeks of gestation and was treated with multiple transfusions of type-speciic packed red blood cells. Shortly thereater, she developed a fever and joint pains, along with laboratory values consistent with hemolysis. Timing suggested a delayed transfusion reaction. An extensive evaluation including red blood cell antigen identiication and cross-reaction failed to reveal the cause for her hemolysis. Despite her critically low hemoglobin levels, her transfusions were withheld in an attempt to allow the patient to recover conservatively. With this strategy, her hemoglobin remained below her baseline, but her symptoms began to improve. Her laboratory values normalized, and hemolysis was no longer evident. hree weeks later, her hemoglobin levels returned back to her baseline without additional intervention. She went on to deliver a full-term male infant. 1. Introduction Worldwide, approximately 270 million people are estimated to be either silent carriers or directly afected by a hemoglobin disorder. he incidence is on the rise, most notably in North America, relecting a shit in the epidemiology [1]. Changes in population demographics due to increased ethnic integration have resulted in a higher number of infants born with a hemoglobinopathy, thus increasing the number of cases encountered in obstetrical practice [2]. his trend in shiting demographics has contributed to the increased complexity of these disorders, making them more diicult to manage. Whereas historical carriers would possess alterations on a single gene, patients with a variety of permutations are now becoming more frequent, resulting in a number of new phenotypes and challenging traditional guidelines [3, 4]. Treatment is especially challenging when patients become pregnant, as the physiologic demands of pregnancy place a larger burden on hemoglobin reserves. Pregnancy itself has also been identiied as a known risk factor for developing hemolytic crises in patients with severe hemoglobinopathies [3, 5]. he American Society of Hematology currently rec- ommends red blood cell transfusion to maintain patient’s hemoglobin above 8.0-9.0g/dL in patients with severe ane- mias [3]. For pregnant patients, many institutions employ more aggressive protocols aimed at maintaining hemoglobin levels above 10.0 g/dL and using transfusions as the irst- line response to sustain adequate levels [4, 6, 7]. However, transfusions always carry risks, both immediate and delayed. A hemolytic transfusion reaction is deined as the rapid destruction of red blood cells following a transfusion, and it occurs, in the United States, in 1 out of every 40,000 trans- fused units with incidence higher for those with repeat trans- fusions [6, 8]. We present a case of a severe hemoglobinopa- thy in pregnancy that was complicated by acute hemolysis and ultimately diagnosed as a delayed transfusion reaction. Conventional therapy produced worsening disease, and the patient was ultimately treated by withholding blood products. his case highlights the diiculty in treating pregnant patients with severe hemoglobinopathies, the need to consider other