Donath-Landsteiner Hemolytic Anemia Clinical Presentation

  • Author: Trisha Simone Tavares, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 6, 2012
 

History

The classic symptoms of Donath-Landsteiner hemolytic anemia (DLHA) include a sudden onset of hemoglobinuria accompanied by pallor and mild jaundice.[13] Hemoglobinuria may be absent if the autoantibody level is not high enough to cause intravascular hemolysis.

A preceding history of upper respiratory tract infection or other viral infection is common, particularly in the younger age group. Preceding exposure to cold may be elicited on the patient history.[3] It is not consistently reported, however. In fact, only 1 in 52 cases of DLHA reported by Sokol et al occurred after documented exposure to cold.[8]

Anemia may be mild, moderate, or severe. Approximately two thirds of patients require RBC transfusion.

High fevers, chills, back or leg pain, and abdominal cramping may also be reported, along with headache, nausea, vomiting, and diarrhea. These symptoms typically resolve early in the clinical course.

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Physical Examination

Symptoms of anemia include the following:

  • Pallor
  • Tachycardia
  • Fatigue
  • Dyspnea
  • Cardiac murmur

Symptoms of hemolysis include the following:

  • Jaundice
  • Dark urine caused by hemoglobinuria

Systemic symptoms include the following:

  • Fever, rigors
  • Pain or cramping in the back, legs, or abdomen
  • Headache
  • Nausea, vomiting, diarrhea

Vasomotor symptoms include the following:

Hepatosplenomegaly may be noted in approximately one fourth of patients.

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Contributor Information and Disclosures
Author

Trisha Simone Tavares, MD  Assistant Professor of Pediatrics, Attending Physician, Department of Pediatrics, Section of Hematology and Oncology, Center for Children's Cancer and Blood Disorders, Golisano Children's Hospital, State University of New York Upstate Medical University; Attending Physician, Department of Pediatrics, Crouse Hospital

Trisha Simone Tavares, MD is a member of the following medical societies: Children's Oncology Group and Medical Society of the State of New York

Disclosure: Nothing to disclose.

Coauthor(s)

M Monica Gramatges, MD  Assistant Professor, Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine

M Monica Gramatges, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Children's Oncology Group

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Nicolas A Camilo, MD Consulting Staff, Division of Pediatric Hematology-Oncology, Mountain States Tumor Institute, St Luke's Regional Medical Center

Nicolas A Camilo, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Gary D Crouch, MD Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Michael R Jeng, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology, Stanford University School of Medicine

Michael R Jeng, MD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Histiocyte Society

Disclosure: Nothing to disclose.

Gary R Jones, MD Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Thomas W Loew, MD Clinical Professor of Pediatrics, Division Director of Pediatric Hematology/Oncology, University of Missouri Children's Hospital

Thomas W Loew, MD is a member of the following medical societies: American Academy of Pediatrics, American Academy of Pediatrics, American College of Physician Executives, American Medical Association, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Children's Oncology Group

Disclosure: Genzyme Grant/research funds Independent contractor; Genzyme Honoraria Speaking and teaching; Amicus Grant/research funds Independent contractor; Purdue Pharmaceuticals Grant/research funds Independent contractor

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References

  1. Donath J LK. Uber paroxysmale Hamoglobinurie. Munchener Medizinische Wochenschrift. 1904;51:1590-3.

  2. Bird GW. Paroxysmal cold haemoglobinuria. Br J Haematol. Oct 1977;37(2):167-71. [Medline].

  3. Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol. Apr 2002;69(4):258-71. [Medline].

  4. Sivakumaran M, Murphy PT, Booker DJ, Wood JK, Stamps R, Sokol RJ. Paroxysmal cold haemoglobinuria caused by non-Hodgkin's lymphoma. Br J Haematol. Apr 1999;105(1):278-9. [Medline].

  5. Sharara AI, Hillsley RE, Wax TD, Rosse WF. Paroxysmal cold hemoglobinuria associated with non-Hodgkin's lymphoma. South Med J. Mar 1994;87(3):397-9. [Medline].

  6. Lippman SM, Winn L, Grumet FC, Levitt LJ. Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria. Am J Med. May 1987;82(5):1065-72. [Medline].

  7. Petz LD. Cold antibody autoimmune hemolytic anemias. Blood Rev. Jan 2008;22(1):1-15. [Medline].

  8. Sokol RJ, Booker DJ, Stamps R. Erythropoiesis: Paroxysmal Cold Haemoglobinuria: A Clinico-Pathological Study of Patients with a Positive Donath-Landsteiner Test. Hematology. 1999;4(2):137-164. [Medline].

  9. Sokol RJ, Hewitt S, Stamps BK. Autoimmune haemolysis associated with Donath-Landsteiner antibodies. Acta Haematol. 1982;68(4):268-77. [Medline].

  10. Gottsche B, Salama A, Mueller-Eckhardt C. Donath-Landsteiner autoimmune hemolytic anemia in children. A study of 22 cases. Vox Sang. 1990;58(4):281-6. [Medline].

  11. Wolach B, Heddle N, Barr RD, Zipursky A, Pai KR, Blajchman MA. Transient Donath-Landsteiner haemolytic anaemia. Br J Haematol. Jul 1981;48(3):425-34. [Medline].

  12. Taylor CJ, Neilson JR, Chandra D, Ibrahim Z. Recurrent paroxysmal cold haemoglobinuria in a 3-year-old child: a case report. Transfus Med. Oct 2003;13(5):319-21. [Medline].

  13. Heddle NM. Acute paroxysmal cold hemoglobinuria. Transfus Med Rev. Jul 1989;3(3):219-29. [Medline].

  14. Hernandez JA, Steane SM. Erythrophagocytosis by segmented neutrophils in paroxysmal cold hemoglobinuria. Am J Clin Pathol. Jun 1984;81(6):787-9. [Medline].

  15. Win N, Stamps R, Knight R. Paroxysmal cold haemoglobinuria/Donath-Landsteiner test. Transfus Med. Jun 2005;15(3):254. [Medline].

  16. [Guideline] Gibson BE, Todd A, Roberts I, et al. Transfusion guidelines for neonates and older children. Br J Haematol. Feb 2004;124(4):433-53. [Medline]. [Full Text].

  17. Koppel A, Lim S, Osby M, Garratty G, Goldfinger D. Rituximab as successful therapy in a patient with refractory paroxysmal cold hemoglobinuria. Transfusion. Oct 2007;47(10):1902-4. [Medline].

  18. Roy-Burman A, Glader BE. Resolution of severe Donath-Landsteiner autoimmune hemolytic anemia temporally associated with institution of plasmapheresis. Crit Care Med. Apr 2002;30(4):931-4. [Medline].

 
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Blood smear showing spherocytosis, polychromatophilia, and erythrophagocytosis by neutrophils.
Blood smear showing spherocytosis, polychromatophilia, and erythrophagocytosis by neutrophils.
 
 
 
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