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Hematol Oncol Clin North Am. 2015 Jun;29(3):473-8. doi: 10.1016/j.hoc.2015.01.004. Epub 2015 Mar 7.

Paroxysmal cold hemoglobinuria.

Author information

1
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.
2
Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, Suite 4500, 301 building 4940 Eastern Ave, Baltimore, MD 21224, USA.

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.

KEYWORDS:

Autoimmune hemolytic anemia; Coombs negative hemolysis; Donath-Landsteiner; PCH; Syphilis

PMID:
26043386
DOI:
10.1016/j.hoc.2015.01.004
[Indexed for MEDLINE]

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