Hot and Cold

  • Mark T. Friedman
  • Kamille A. West
  • Peyman Bizargity
  • Kyle Annen
  • Jeffrey S. Jhang


A 5-year-old boy presents to the emergency department (ED) with 2 days of fever (temperature 100.7 °F), fatigue, jaundice, and dark urine. The parents note that the child had symptoms of a viral upper respiratory illness 2 weeks before presentation and that he was previously healthy with no history of blood transfusion. In the ED, the child weighs 21.3 kg, and laboratory workup reveals severe anemia with hemoglobin (Hgb) 5.4 g/dL, elevated total bilirubin 5.1 mg/dL, aspartate aminotransferase (AST) 357 U/L (normal range, 8–60 U/L), and lactate dehydrogenase (LDH) 8126 U/L. Serum haptoglobin is undetectable. Urinalysis results are as follows: color, dark; red blood cells (RBCs), 0–3 per high-power field; and urine Hgb, 3+ positive. Notably, the onset of symptoms (fever, fatigue, jaundice, dark urine) was associated with eating a bowl of ice cream. An ethylenediaminetetraacetic acid (EDTA) anticoagulant type and screen sample is submitted to the blood bank along with a request for RBC transfusion.


Autoimmune hemolytic anemia Biphasic hemolysin Donath-Landsteiner P antigen Paroxysmal cold hemoglobinuria/PCH 


  1. 1.
    Salido EJ, Cabañas V, Berenguer M, Macizo MI, García-Candel F, Pérez-López R, Moraleda JM. Serological findings in a child with paroxysmal cold haemoglobinuria. Case Rep Med. 2014;2014:Article ID 316010. Accessed 3 Mar 2018.CrossRefGoogle Scholar
  2. 2.
    Vaglio S, Arista MC, Perrone MP, Tomei G, Testi AM, Coluzzi S, Girelli G. Autoimmune hemolytic anemia in childhood: serologic features in 100 cases. Transfusion 2007;47(1):50–54.CrossRefGoogle Scholar
  3. 3.
    Eder AF. Review: acute Donath-Landsteiner hemolytic anemia. Immunohematology. 2005;21(2):56–62.PubMedGoogle Scholar
  4. 4.
    Leger RM. The positive direct antiglobulin test and immune-mediated hemolysis. In: Fung MK, Grossman BJ, Hillyer CD, Westhoff CM, editors. Technical manual. 18th ed. Bethesda: AABB; 2014. p. 440.Google Scholar
  5. 5.
    Koppel A, Lim S, Osby M, Garratty G, Goldfinger D. Rituximab as successful therapy in a patient with refractory paroxysmal cold hemoglobinuria. Transfusion. 2007;47(10):1902–4.CrossRefGoogle Scholar
  6. 6.
    Prince SD, Winestone LE, Nance SJ, Friedman DF. Recurrent Donath-Landsteiner hemolytic anemia: a pediatric case report. Transfusion. 2017;57(6):1401.CrossRefGoogle Scholar

Recommended Reading

  1. Donath J, Landsteiner K. Űber paroxysmale haemoglobinurie. Műnchen Medicine Wochenschr. 1904;51:1590–3.Google Scholar
  2. Eder AF. Review: acute Donath-Landsteiner hemolytic anemia. Immunohematology. 2005;21(2):56–62.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Mark T. Friedman
    • 1
  • Kamille A. West
    • 2
  • Peyman Bizargity
    • 3
  • Kyle Annen
    • 4
  • Jeffrey S. Jhang
    • 1
  1. 1.Icahn School of MedicineMount Sinai Health SystemNew YorkUSA
  2. 2.Department of Transfusion MedicineNational Institutes of Health Clinical CenterBethesdaUSA
  3. 3.Department of Molecular & Human GeneticsBaylor College of MedicineHoustonUSA
  4. 4.Department of PathologyChildren’s Hospital ColoradoAuroraUSA

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