The Anesthetic Management of a Patient with Paroxysmal Nocturnal Hemoglobinuria

Kathirvel, S.; Prakash, Avinash; Lokesh, B.; Sujatha, P

doi:10.1097/00000539-200010000-00050

Cited by 15 publications

(8 citation statements)

References 12 publications

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“…As perioperative care in PNH patients, the prevention of hemolytic attacks due to surgical invasion and thrombosis associated with increased activity in the coagulation system is important. Conventionally, the pre- and postoperative administration of heparin calcium or dalteparin sodium was recommended as perioperative care [ 8 , 9 ]. To prevent hemolytic attacks, the use of steroids was also recommended [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

A patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case

et al. 2015

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Paroxysmal nocturnal hemoglobinuria (PNH) is acquired hemolytic anemia characterized by symptoms such as anemia and hemoglobinuria. In recent years, eculizumab as an anti-complement (C5) monoclonal antibody has been used for PNH and shown to have marked effects. We performed laparoscopic cholecystectomy in a patient with PNH being treated with eculizumab, and could avoid the risk of perioperative hemolysis and thrombosis. [Patient] The patient was a 48-year-old female who had developed PNH when she was 39 years old. At the age of 46 years, eculizumab administration was initiated once every 2 weeks. During the administration period, neither the progression of anemia nor hemoglobinuria was observed. In March 2013, gallstones were detected, and she was referred to our hospital for surgery. Eculizumab was administered 10 days before surgery, and laparoscopic cholecystectomy was performed in May 2013. After the operation, for the prevention of thrombosis, elastic stockings and a foot pump were used without anticoagulant administration. After the operation, neither the progression of anemia nor hemoglobinuria was observed. On postoperative day 5, eculizumab was administered as planned, and she showed a favorable general condition and was discharged. [Discussion] Perioperative care in PNH patients was conventionally considered to involve a high risk of developing anemia, thrombosis, or infection. However, after the advent of eculizumab, the control of the symptoms of PNH became possible in many patients. In this patient with PNH being treated with eculizumab, safe perioperative management was possible without the development of complications.

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Section: Discussionmentioning

confidence: 99%

A patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case

et al. 2015

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“…Surgery is one of the major risk factors triggering hemolytic crisis and thrombosis in PNH through C 0 activation [3], the various mechanisms of which include the physiological stress response to surgical procedures via inflammatory mediators [14] and anesthesia, which induces many factors predisposing acidosis, such as hypoxemia, hypoperfusion, and hypercapnia [15]. 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].…”

Section: Discussionmentioning

confidence: 99%

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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“…Washing red cells is no longer felt to be necessary and was historically recommended on the basis of ABO isoagglutinins in group O whole blood that caused dramatic haemolysis in a group A recipient (Brecher & Taswell, 1989). Kathirvel et al (2000) recommend that PNH patients should be transfused with ABO-identical fresh blood and blood products. The definition of 'fresh' blood has recently been standardized in New Zealand to red cells stored for Table 1.…”

Section: Discussionmentioning

confidence: 99%

Cardiopulmonary bypass surgery in a patient with paroxysmal nocturnal haemoglobinuria

D¹,

Baker

Carter

2006

Transfusion Medicine

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Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired haemolytic anaemia. Cardiac bypass surgery in patients with PNH may be complicated by aggravation of haemolysis in addition to increased risk of infection, renal failure, bleeding and thrombosis. We describe a case of uncomplicated cardiopulmonary bypass surgery for aortic valve replacement and aortoplasty in a patient with PNH and discuss some recommendations to reduce the risk of peri-operative complications.

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The Anesthetic Management of a Patient with Paroxysmal Nocturnal Hemoglobinuria

Abstract: This case report describes the anesthetic considerations for a patient with paroxysmal nocturnal hemoglobinuria. Specific strategies to be applied in the perioperative period to prevent hemolytic episodes and venous thrombosis are also discussed.

Cited by 15 publications

References 12 publications

A patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case

A patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case

Perisurgical induction of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria

Cardiopulmonary bypass surgery in a patient with paroxysmal nocturnal haemoglobinuria

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