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

Abstract

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.

Cholecystectomy in a patient with paroxysmal nocturnal haemoglobinuria undergoing ravulizumab maintenance treatment

“…Patients with PNH are at a high risk of complement‐mediated IVH and thrombosis during or after surgery. Successful cases of cardiopulmonary bypass surgery and cholecystectomy have been reported in patients with PNH during eculizumab treatment [ 7 , 8 , 9 , 10 , 11 ]. These reports suggest that C5 inhibitor treatment prevents surgery‐induced BTH and thrombosis in patients with PNH.…”

An unusual postoperative manifestation of paroxysmal nocturnal haemoglobinuria following gender re‐affirming mastectomy

“…Similar cases of cutaneous thrombosis and hemorrhagic necrosis have been described, in patients who display areas of necrotic, violaceous plaques with bullous, haemorrhagic features, 6,7 where surgery precipitates episodes of haemolysis. Moriyama et al 8 proposed judicious perioperative administration of eculizumab was able to prevent the potential complications of PNH after laparoscopic cholecystectomy. Our case had also had regular eculizumab administered perioperatively, under close supervision by the haematology unit of the treating hospital.…”

How to apply clinical cases and medical literature in the framework of a modified “failure mode and effects analysis” as a clinical reasoning tool – an illustration using the human biliary system

“…Excessive hemolysis leads to increase in unconjugated bilirubinJaundice; generalized tonic–clonic seizure when kernicterus occurs [82]Liver function test, serum G6PD level, G6PD deficiency phenotyping [82]G6PD deficiency is an X-linked recessive diseaseParoxysmal nocturnal hemoglobinuriaProlonged and recurrent red blood cell breakdown results in increased bilirubin in the gallbladder. The excess bilirubin can precipitate bilirubin stones.Gallstones in the common bile duct [83]Jaundice and abdominal pain [83]CT, ERCP, MRCP [83]Red blood cell membrane defect resulting in spherical, osmotically fragile erythrocytes (hereditary spherocytosis) [84]Premature red blood cell destruction leading to hyperbilirubinemia [84]Anemia, jaundice, and splenomegaly [84]Peripheral blood smear showed small and dense spherocytes; osmotic fragility test [84]The patient in the case has coexistence of hereditary spherocytosis and Gilbert syndrome [84]Endocrinological processPoorly controlled diabetes mellitus [85]The excess glucose increases glycogen storage in the liver and blocks glycogenolysis resulting in glycogenic hepatopathy [85]Hepatomegaly and pain in the RUQ [85]Ultrasound abdomen, liver function test, and liver biopsy [85]This table is neither exhaustive nor comprehensive. For instance, it has not included drug-induced liver injury, and various infective causes such as hepatitis.…”