Cardiopulmonary bypass surgery in a patient with paroxysmal nocturnal haemoglobinuria

D, Dinesh Roy; Baker, Bart; Carter, J. M.

doi:10.1111/j.1365-3148.2006.00668.x

Cited by 13 publications

(12 citation statements)

References 7 publications

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“…Drug therapies, such as steroid and androgen therapies, constitute the conventional management, but they only afford the suppression of hemolysis and the improvement of hematopoiesis, and also remain controversial in regard to their effectiveness (3,4). To date, there is no curative treatment for patients with PNH, except for allogeneic bone marrow transplantation.…”

Section: Introductionmentioning

confidence: 99%

Successful Management of Obstructive Jaundice due to Gallstones with Eculizumab in a Patient with Paroxysmal Nocturnal Hemoglobinuria

et al. 2012

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Paroxysmal nocturnal hemoglobinuria (PNH) makes patients susceptible to intravascular hemolysis and thrombosis, and it can be life-threatening in stressful situations. Eculizumab, a humanized monoclonal antibody that inhibits the complement protein C5, has been evaluated as a novel therapy for PNH. We herein describe the case of a 59-year-old Japanese woman with classic PNH, who had been successfully treated with eculizumab, but who later developed acute cholecystitis/cholangitis from gallstones. Although the severe obstructive jaundice requiring endoscopic therapy following cholecystectomy was complicated, critical intravascular hemolysis and thrombosis were not observed. Therefore, utilizing eculizumab during the peri-operative management of PNH patients should be carefully taken into consideration.

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Section: Introductionmentioning

confidence: 99%

Successful Management of Obstructive Jaundice due to Gallstones with Eculizumab in a Patient with Paroxysmal Nocturnal Hemoglobinuria

et al. 2012

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“…In order to reduce the risk of perioperative hemolysis in PNH, RBC transfusion has been performed in several reports, which can decrease the proportion of PNH-RBCs [14,16,17]. We did not use prophylactic heparin in this case because significant risk of hemorrhage is expected around the time of surgery, and because thrombosis is less prevalent in Japanese patients than white patients [18].…”

Section: Discussionmentioning

confidence: 73%

Perisurgical induction of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria

Naoki

Obara

Fukuda

et al. 2013

Blood Coagulation &Amp; Fibrinolysis

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Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by complement (C')-induced lysis of PNH red blood cells (RBCs), which are deficient in the expression of CD55 and CD59. Surgery is one of the major clinical situations that trigger hemolytic attack and thrombosis in PNH. We describe here a case of 64-year-old man with classic PNH complicated by early-stage gastric cancer requiring distal gastrectomy under general anesthesia. We administered humanized monoclonal anti-C5 antibody (eculizumab; Soliris) for a limited period (600 mg, once a week × four times) perisurgically. Eculizumab effectively inhibited the C' system and the patient underwent a curative distal gastrectomy without significant surgery-triggered hemolytic attack. Although discontinuation of eculizumab induced mild hemolysis 2 weeks after the last administration, it was treated conservatively without thrombotic complication. Limited-term induction of eculizumab could be an option for PNH patients with transient and anticipated high risks, with careful preparation for the discontinuation-related risks afterwards.

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“…[1][2][3] This arises from a somatic mutation of the X-chromosomal phosphatidylinositol glycan complementation class A gene that renders PNH erythrocytes abnormally susceptible to complement-mediated attack, thereby shortening the survival of these cells in vivo to 10% of that of normal cells. 2,3 Patients with PNH have persistent low levels of hemolysis; however, they can have acute exacerbations resulting in acute hemolytic crisis.…”

Section: Discussionmentioning

confidence: 99%

Cardiac surgery in a patient with paroxysmal nocturnal hemoglobinuria

Chu

Andrews

Watanabe

2011

Asian Cardiovasc Thorac Ann

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A 44-year-old woman, who had been diagnosed with paroxysmal nocturnal hemoglobinuria, presented with chest pain. Angiography revealed critical distal left main stenosis and proximal stenoses of the left anterior descending and circumflex arteries. Prophylactic antibiotics, infusion of blood products and mannitol intraoperatively, and antithrombotic therapy postoperatively ensured a good outcome of coronary artery bypass graft surgery under cardiopulmonary bypass.

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Cardiopulmonary bypass surgery in a patient with paroxysmal nocturnal haemoglobinuria

Cited by 13 publications

References 7 publications

Successful Management of Obstructive Jaundice due to Gallstones with Eculizumab in a Patient with Paroxysmal Nocturnal Hemoglobinuria

Successful Management of Obstructive Jaundice due to Gallstones with Eculizumab in a Patient with Paroxysmal Nocturnal Hemoglobinuria

Perisurgical induction of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria

Cardiac surgery in a patient with paroxysmal nocturnal hemoglobinuria

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