Paroxysmal cold hemoglobinuria due to an IgA Donath-Landsteiner antibody

Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic anemia (AIHA) characterized by the presence of a Donath–Landsteiner (D‐L) antibody. PCH occurs most commonly in young children and is associated with acute, often self‐limited hemolytic anemia. The D‐L antibody is classically a biphasic...

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Veröffentlicht in:Pediatric blood & cancer 2015-11, Vol.62 (11), p.2044-2046
Hauptverfasser: Whipple, Nicholas S., Moreau, Dawn AB, Moulds, JoAnn M., Hankins, Jane S., Wang, Winfred C., Nottage, Kerri A.
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Sprache:eng
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Zusammenfassung:Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic anemia (AIHA) characterized by the presence of a Donath–Landsteiner (D‐L) antibody. PCH occurs most commonly in young children and is associated with acute, often self‐limited hemolytic anemia. The D‐L antibody is classically a biphasic IgG anti‐P autoantibody identified by the D‐L test. Rare case reports confirm the existence of IgM D‐L antibodies. We report the case of a 2‐year‐old male diagnosed with acute AIHA and found to have PCH caused by an IgA D‐L antibody. The clinical course and treatment of this condition, which has not been reported previously, are described. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.25591