| Autor: |
Jochmans, Kristin, Vandenbroucke, F., De Waele, Marc, Trullemans, Fabienne |
| Přispěvatelé: |
Hematology, Supporting clinical sciences, Medical Imaging, Radiology, Reproductive immunology and implantation, Clinical sciences |
| Jazyk: |
angličtina |
| Rok vydání: |
2011 |
| Předmět: |
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| Popis: |
Paroxysmal nocturnal haemoglobinuria (PHN)is a clonal haematopoietic stem cell disorder. It results from an acquired somatic mutation in the PIG-A gene, causing deficient synthesis of glycosylphosphatidyl-inositol, a cell membrane anchor for diffent proteins. The disease is characterised by complement-mediated intravascular haemolysis, thromboembolism and cytopenia. Thrombosis remains the leading cause of death. Pregnancy in PNH is associated with increased risk of maternal and fetal morbidity and mortality and therefore is discouraged. We report the case of a 22-year-old woman with PNH presenting at 31 weeks of gestation with severe haemolytic anaemia and thrombocytopenia, requiring weekly transfusions. Thromboprophylaxis with nadroparin (6000 IU/day) has been prescribed since about 15 weeks. A 36 weeks she goes into spontaneous labour and is delivered by Caesarean section. Thromboprophylaxis is intensified to therapeutic doses of LMWH. On day 3 postpartum CT scan shows a large floating thrombus in the inferior vena cava and complete thrombosis of the portal vein. Unfractionated heparin is started in continuous infusion. Since eculizumab, a humanised monoclonal antibody directed against complement protein C5 has recently revolutionised treatment in PNH, she is started on this therapy on day 5. Despite full anticoagulation, she develops pulmonary emboli and splenic infarction on day 15. One day later splenic rupture leads to acute haemorrhagic shock. A caval filter is placed and splenectomy is performed in emergency. Her condition stabilises progressively and 10 days after starting eculizumab haemolysis declines strikingly. No further thromboembolic events occur. The use of eculizumab in the postpartum period seems to have the potential benefit of blocking thromboembolic complications in very high risk patients. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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