Report on a patient with paroxysmal cold hemoglobinuria

Int J Hematol. 1997 Feb;65(2):165-7. doi: 10.1016/s0925-5710(96)00548-8.

Abstract

Antibodies against the human blood group P antigen (anti-P alloantibodies) agglutinate phenotype P1 and P2 erythrocytes treated with papain at 4 degrees C but not phenotype PK and p erythrocytes. This condition is referred to as an autoimmune disease of paroxysmal cold hemoglobinuria (PCH), and the anti-P specificity is found in the cold hemagglutinins. Serum from a patient suspected to be suffering from PCH by the cold auto-agglutination properties was tested for anti-P specificity, using papain-treated O blood group erythrocytes. The serum-mediated hemagglutination and the serum and complement-mediated immunohemolysis were inhibited by globoside (P antigen GalNAc beta 1-3Gal alpha 1-4Gal beta 1-4Glc-ceramide) and Forssman glycosphingolipid (GalNAc alpha 1-3GalNAc beta 1-3Gal alpha 1-4Gal beta 1-4Glc-ceramide). Therefore, we concluded that she had PCH. She was completely cured 6 months later.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Globosides / immunology*
  • Hemoglobinuria, Paroxysmal / blood
  • Hemoglobinuria, Paroxysmal / immunology*
  • Hemoglobinuria, Paroxysmal / physiopathology
  • Humans
  • Isoantibodies / blood*
  • Middle Aged

Substances

  • Globosides
  • Isoantibodies