Thrombotic microangiopathy in pregnancy complicated by acute hemorrhagic-necrotic pancreatitis during early puerperium

Summary

Objective:
Authors in the article describe a case of a patient with thrombotic thrombocytopenic purpurain 37 weeks gestation complicated by acute severe hemorrhagic-necrotic pancreatitis during the early puerperium. Design: Case report. Setting: Ist Department of gynaecology and obstetrics of the Comenius University Bratislava. Case-report: 33-years-old patient in the 37 weeks gestation was admitted to our department with the signs of HELLP syndrome (hemolysis, elevated liver enzymes, low platelets). Due to the worsening clinical status, we have performed caesarean section. After the transient stabilization of the patient´s clinical status, the hemolysis with severe thrombocytopenia reappeared. Based on the clinical signs of abdominal pain and computer tomography, the diagnosis of acute hemorrhagic-necrotic pancreatitis was set. The primary diagnosis was thrombotic thrombocytopenic purpura. Therefore, therapeutic plasma exchange was performed with consequent improvement of the patient’s clinical state. Normalization of the platelet count was achieved after 4.plasma exchanges. Consequently 5 plasma exchanges were performed. However, one month later, the disease relapsed. Therapeutic plasma exchanges were needed again (4x), with anti CD 20 administration. This therapy had good clinical outcome, without the need for further plasma exchanges. Conclusion: Thrombotic thrombocytopenic purpura is highly lethal disease. Early diagnosis, treatment, and multidisciplinary approach are essential.. Keywords: HELLP syndrome, thrombotic thrombocytopenic purpura, acute pancreatitis