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Rotational Thromboelastometry (ROTEM)-Assisted Anaesthetic Management of a Parturient With Paroxysmal Nocturnal
Despoina Fani Papadaki1, Emmanouil Stamatakis2, Giolanda Varvarousi2
1Department of Anaesthesiology, Hellenic Red Cross Hospital "Korgialeneio-Benakeio", Athens, GRC.
Abstract:
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired, clonal haematopoietic disorder. It is life-threatening and is characterized by intravascular haemolytic anaemia, large vessel thrombosis and bone marrow failure. Aplastic anaemia (AA) and PNH are intimately linked; however, concurrent treatment of PNH and AA is uncommon. Pregnancies in patients with PNH are rare and associated with increased maternal and fetal morbidity and mortality. Nevertheless, with the advent of eculizumab, a monoclonal antibody used to treat PNH, pregnancy outcomes have become more favourable. The obstetric and anaesthetic management of affected patients poses significant challenges, making multidisciplinary involvement essential. We report the successful management of a parturient with PNH and severe AA who underwent a scheduled caesarean section.
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