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Clinical Management of Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Case Report and Updated Review

Fieni, Stefania MD*; Bonfanti, Laura MD; Gramellini, Dandolo MD; Benassi, Luigi MD; Delsignore, Roberto MD§

Obstetrical & Gynecological Survey: September 2006 - Volume 61 - Issue 9 - p 593-601
doi: 10.1097/01.ogx.0000234794.27485.59
CME Program: CATEGORY 1 CME REVIEW ARTICLES 25, 26, AND 27: CME REVIEW ARTICLE 25

Because women with paroxysmal nocturnal hemoglobinuria (PNH) are especially vulnerable to thromboembolic phenomena, pregnancy is a time of increased risk for both mother and fetus. However, pregnancies in affected women are rare; only case reports and small studies have been reported so far. We present the case of a 20-year-old woman with PNH who, while undergoing medical tests in preparation for a bone marrow transplant, was discovered to be pregnant. We also review the obstetric literature on pregnancy complicated by PNH, which indicates that both maternal and fetal mortality is exceptionally high (11.6% and 7.2%) with the major cause of maternal mortality being thromboembolism. Major maternal complications are more frequent postpartum (30.2%) than antepartum or intrapartum (16.3%).

Target Audience: Obstetricians & Gynecologists, Family Physicians

Learning Objectives: After completion of this article, the reader should be able to recall that paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy increases adverse events for both the mother and the fetus, state that maternal and fetal mortality are both high, and explain that the major complications occur in the postpartum period.

*Researcher, Gynecology, Obstetrics and Neonatology Department; †Medical Doctor, Internal Medicine and Biomedical Sciences Departments; ‡Associate Professor, Gynecology, Obstetrics and Neonatology Department; and §Director, Internal Medicine and Biomedical Sciences Departments, University of Parma, Parma, Italy

Chief Editor’s Note: This article is part of a series of continuing education activities in this Journal through which a total of 36 AMA/PRA category 1 credits™ can be earned in 2006. Instructions for how CME credits can be earned appear on the last page of the Table of Contents.

The authors have disclosed that they have no financial relationships with or interests in any commercial companies pertaining to this educational activity.

The authors have disclosed that eculizumab has not been approved by the U.S. Food and Drug Administration.

Lippincott Continuing Medical Education Institute, Inc. has identified and resolved all faculty conflicts of interest regarding this educational activity.

Reprint requests to: Dandolo Gramellini, MD, Department of Obstetrics, Gynecology and Neonatology, University Hospital, Via Gramsci, 14, 43100 Parma, Italy. E-mail: dandolo.gramellini@unipr.it.

© 2006 Lippincott Williams & Wilkins, Inc.