Keimyung Med J.  2018 Dec;37(2):92-96. 10.0000/kmj.2018.37.2.92.

Anesthetic and Perioperative Management of Patient with Paroxysmal Nocturnal Hemoglobinuria

• Choi EK
• Kim KB
• Park SJ
• Park KB

• KMID: 2437309
• DOI: http://doi.org/10.0000/kmj.2018.37.2.92

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematopoietic stem cell disorder. Surgery, anesthesia, infection, trauma, and pregnancy can exacerbate hemolysis and thrombotic risk by increasing complement activity. However, perioperative treatment of eculizumab can reduce risk of hemolytic event by surgical stress. Here, we present the perioperative clinical course and adequate anesthetic management of PNH complicated by spine surgery under general anesthesia. We also describe a possible role of eculizumab for PNH patients with anticipated high risks.

Keyword

Hemoglobinuria; Hemolysis; Paroxysmal; Thrombosis

MeSH Terms

Anesthesia
Anesthesia, General
Complement System Proteins
Hematopoietic Stem Cells
Hemoglobinuria
Hemoglobinuria, Paroxysmal*
Hemolysis
Humans
Pregnancy
Spine
Thrombosis
Complement System Proteins