Paroxysmal cold hemoglobinuria

Paroxysmal cold hemoglobinuria
Other namesDonath-Landsteiner syndrome
SpecialtyHematology

Paroxysmal cold hemoglobinuria (PCH) or Donath–Landsteiner hemolytic anemia (DLHA) is an autoimmune hemolytic anemia featured by complement-mediated intravascular hemolysis after cold exposure.[1] It can present as an acute non-recurrent postinfectious event in children, or chronic relapsing episodes in adults with hematological malignancies or tertiary syphilis. Described by Julius Donath (1870–1950) and Karl Landsteiner (1868–1943) in 1904, PCH is one of the first clinical entities recognized as an autoimmune disorder.[2]

Paroxysmal cold hemoglobinuria is a result of cold-reacting antibod immunoglobulin (Ig) induced hemolytic response inside vessels leading to anemia[3] and, thus, a cold antibody autoimmune hemolytic anemias (CAAHA).[3]

In most patients with DLHA, the antibody selectively targets against the red blood cells on-surface antigen called the antigen P or antigen I, respectively.[3] Most cases were found to be owing to polyclonal IgG. Nonetheless, IgM-induced DLHA has already also been described in the past.[3][4][5][6] For example, there was a case study reporting that autoimmune hemolytic anemia where an IgA Donath–Landsteiner denoted as [D-L] antibody appeared to cause Donath–Landsteiner cold hemoglobinuria.[7] The most notable difference between DLHA and CAD (cold agglutinin disease) is the causative agent. For cold agglutinin disease, the causative agent is constantly owing to a cold-active IgM antibody.[3]

  1. ^ Shanbhag, Satish; Spivak, Jerry (June 2015). "Paroxysmal Cold Hemoglobinuria". Hematology/Oncology Clinics of North America. 29 (3): 473–478. doi:10.1016/j.hoc.2015.01.004. ISSN 0889-8588. PMID 26043386.
  2. ^ Moticka, Edward J. (2013). Historical perspective on evidence-based immunology. Elsevier Science Publishing. p. 300. ISBN 9780123983817.
  3. ^ a b c d e Trisha Simone Tavares, MD, FAAP Attending Physician, Department of Pediatrics, Section of Hematology/Oncology, Cardon Children's Medical Center. Trisha Simone Tavares, MD, FAAP is a member of the following medical societies: Children's Oncology Group (2019-02-02). "Donath–Landsteiner Hemolytic Anemia: Practice Essentials, Pathophysiology, Etiology". Medscape Reference. Retrieved 2019-02-11.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  4. ^ Hayashi, Hisako; Yasutomi, Motoko; Hayashi, Taihei; Yuasa, Miori; Kawakita, Akiko; Hata, Ikue; Tanizawa, Akihiko; Muramatsu, Hideki; Kojima, Seiji; Ohshima, Yusei (2013). "Paroxysmal cold hemoglobinuria caused by an IgM-class Donath–Landsteiner antibody". Pediatrics International. 55 (5). Wiley: 664–666. doi:10.1111/ped.12110. ISSN 1328-8067. PMID 24134760. S2CID 206261041.
  5. ^ Karafin, Matthew S.; Shirey, R. Sue; Ness, Paul M.; King, Karen E.; Keefer, Jeffrey (2012-05-02). "A case study of a child with chronic hemolytic anemia due to a Donath–Landsteiner positive, IgM anti-I autoantibody". Pediatric Blood & Cancer. 59 (5). Wiley: 953–955. doi:10.1002/pbc.24185. ISSN 1545-5009. PMID 22553072. S2CID 41343971.
  6. ^ Ogose, Takeshi; Wakata, Yoshifumi; Kaneko, Masaya; Shinahara, Kumi; Takechi, Tomoki; Kotani, Haruko (2007). "A Case of Recurrent Paroxysmal Cold Hemoglobinuria With the Different Temperature Thresholds of Donath–Landsteiner Antibodies". Journal of Pediatric Hematology/Oncology. 29 (10). Ovid Technologies (Wolters Kluwer Health): 716–719. doi:10.1097/mph.0b013e31814d6845. ISSN 1077-4114. PMID 17921855.
  7. ^ Whipple, Nicholas S.; Moreau, Dawn AB.; Moulds, JoAnn M.; Hankins, Jane S.; Wang, Winfred C.; Nottage, Kerri A. (2015-06-05). "Paroxysmal cold hemoglobinuria due to an IgA Donath–Landsteiner antibody". Pediatric Blood & Cancer. 62 (11). Wiley: 2044–2046. doi:10.1002/pbc.25591. ISSN 1545-5009. PMID 26053459. S2CID 45396704.