A Case Report of Budd-Chiari Syndrome in Pregnancy Secondary to Paroxysmal Nocturnal Hemoglobinuria

Dr. Devathi Mounika, Dr. Bindu Vijaykumar

Abstract: Aims: To present a rare and complex case of Budd-Chiari Syndrome (BCS) secondary to previously undiagnosed Paroxysmal Nocturnal Hemoglobinuria (PNH), presenting for the first time during pregnancy, and to highlight the successful management through a multidisciplinary approach. Presentation of Case: A 21-year-old primigravida at 19 weeks of gestation presented with right upper quadrant pain, nausea, and jaundice. Initial investigations revealed severe hemolytic anemia, thrombocytopenia, and deranged liver function. Imaging studies confirmed hepatosplenomegaly, ascites, and extensive thrombosis of the hepatic, portal, and splenic veins, diagnostic of Budd-Chiari Syndrome. Further hematological evaluation, including flow cytometry, identified a large PNH clone, establishing it as the underlying etiology. The patient was managed throughout her pregnancy with anticoagulation, multiple blood transfusions, and close monitoring by a team of obstetricians, hematologists, and gastroenterologists. An elective Caesarean section was performed at 36 weeks, resulting in the delivery of a healthy male infant and a favorable maternal outcome. Discussion: The co-occurrence of PNH and BCS is rare, and its initial presentation during pregnancy poses significant diagnostic and therapeutic challenges. The prothrombotic state of pregnancy likely unmasked the underlying PNH. Management required a delicate balance between preventing further thrombosis and mitigating the risk of hemorrhage, especially during delivery. This case underscores the necessity of considering rare hematological disorders in pregnant patients with atypical presentations of liver disease. Conclusion: Timely diagnosis and collaborative, multidisciplinary management are crucial for achieving successful maternal and fetal outcomes in pregnancies complicated by severe conditions like PNH-induced Budd-Chiari Syndrome.

Keywords: Budd-Chiari Syndrome, Paroxysmal Nocturnal Hemoglobinuria, Pregnancy, Thrombosis, Anticoagulation, Multidisciplinary Care

How to Cite?: Dr. Devathi Mounika, Dr. Bindu Vijaykumar, "A Case Report of Budd-Chiari Syndrome in Pregnancy Secondary to Paroxysmal Nocturnal Hemoglobinuria", Volume 14 Issue 9, September 2025, International Journal of Science and Research (IJSR), Pages: 242-244, https://www.ijsr.net/getabstract.php?paperid=SR25908161715, DOI: https://dx.doi.org/10.21275/SR25908161715