title: Paroxysmal cold hemoglobinuria.
abstract: Paroxysmal cold hemoglobinuria is a rare cause of autoimmune hemolytic anemia predominantly seen as an acute form in young children after viral illnesses and in a chronic form in some hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the P antigen on red cells. The antibody attaches to red cells at colder temperatures and causes red cell lysis when blood recirculates to warmer parts of the body. Treatment is mainly supportive and with red cell transfusion, but immunosuppressive therapy may be effective in severe cases.
authors.affiliation: Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, Suite 4500, 301 building 4940 Eastern Ave, Baltimore, MD 21224, USA. Electronic address: sshanbh2@jhmi.edu.
authors.forename: Satish
authors.initials: S
authors.lastname: Shanbhag
authors.affiliation: Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, Suite 4500, 301 building 4940 Eastern Ave, Baltimore, MD 21224, USA.
authors.forename: Jerry
authors.initials: J
authors.lastname: Spivak
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D003080:Cold Temperature; D003165:Complement System Proteins / Q000276:immunology; D004912:Erythrocytes / Q000276:immunology*; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D006461:Hemolysis / Q000276:immunology; D006801:Humans; D009504:Neutrophils / Q000276:immunology*; D010587:Phagocytosis / Q000276:immunology*
chemical_list: D003165:Complement System Proteins
keywords: Autoimmune hemolytic anemia; Coombs negative hemolysis; Donath-Landsteiner; PCH; Syphilis