eMedicine Specialties > Pediatrics: General Medicine > Hematology

Donath-Landsteiner Hemolytic Anemia: Follow-up

Author: M Monica Gramatges, MD, Fellow in Pediatric Hematology/Oncology, Stanford University
Coauthor(s): Michael R Jeng, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology, Stanford University School of Medicine
Contributor Information and Disclosures

Updated: May 19, 2009

Follow-up

Further Inpatient Care

Indications for hospitalization in patients with Donath-Landsteiner hemolytic anemia (DLHA) include the following:

  • Severe anemia
  • Respiratory or circulatory compromise
  • Renal failure
  • Severe infection

Further Outpatient Care

Perform the following evaluations weekly until the patient is stable and then monthly until Donath-Landsteiner hemolytic anemia resolves:

  • Clinical examination
  • CBC count, total bilirubin, lactate dehydrogenase
  • Reticulocyte count
  • Urinalysis

Perform direct antiglobulin test (DAT) monthly until Donath-Landsteiner hemolytic anemia resolves.

Transfer

  • Transfer patients with severe anemia or complications to a facility where pediatric hematology/oncology, blood bank, and pediatric intensive care services are available.

Deterrence/Prevention

  • Currently, prevention of Donath-Landsteiner hemolytic anemia is not possible.
  • For chronic idiopathic Donath-Landsteiner hemolytic anemia, avoidance of extreme cold exposure may prevent symptoms from occurring.

Prognosis

Prognosis is very good, with most patients recovering within one month of disease onset. Although most cases consist of a single episode, Donath-Landsteiner hemolytic anemia may be recurrent.  One case describing recurrent Donath-Landsteiner hemolytic anemia identified a Donath-Landsteiner (D-L) antibody to an antigen other than anti-P.18

  • Acute Donath-Landsteiner hemolytic anemia is usually transient and self-limiting.
  • Chronic idiopathic Donath-Landsteiner hemolytic anemia is usually mild and responds to general measures, such as avoidance of cold exposure.
  • Chronic syphilis-associated Donath-Landsteiner hemolytic anemia resolves with treatment of syphilis with appropriate antibiotics.

Patient Education

  • Teach patients to observe for symptoms of anemia (eg, dyspnea, palpitations, pallor) and to observe for symptoms of hemolysis (eg, jaundice, dark urine).
  • Instruct patients to avoid exposure to extreme cold if possible as well as to avoid strenuous exercise.

Miscellaneous

Medicolegal Pitfalls

  • The diagnosis of Donath-Landsteiner hemolytic anemia (DLHA) depends on the performance of the specific test to detect the autoantibody, the Donath-Landsteiner (D-L) autoantibody. The clinician must have a high index of suspicion. Donath-Landsteiner hemolytic anemia can be confused with cold agglutinin disease and with warm-antibody autoimmune hemolytic anemia.
  • Limit the use of prednisone to individuals with severe disease. Overuse of prednisone may lead to unnecessary risk.
  • Blood transfusion is indicated only in selected individuals with Donath-Landsteiner hemolytic anemia. Avoid unnecessary transfusions because of the transient nature of the disease. Risks of blood transfusion include transfusion reactions and transmission of infection.

Differential

Anemia, Acute
Anemia, Chronic
Cold Agglutinin Disease
Evans Syndrome
Lymphoproliferative Disorders
Mononucleosis and Epstein-Barr Virus Infection
Non-Hodgkin Lymphoma
Syphilis
Systemic Lupus Erythematosus

 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Thomas W Loew, MD, and Nicolas A Camilo, MD, to the original writing and development of this article.



More on Donath-Landsteiner Hemolytic Anemia

Overview: Donath-Landsteiner Hemolytic Anemia
Differential Diagnoses & Workup: Donath-Landsteiner Hemolytic Anemia
Treatment & Medication: Donath-Landsteiner Hemolytic Anemia
Follow-up: Donath-Landsteiner Hemolytic Anemia
Multimedia: Donath-Landsteiner Hemolytic Anemia
References
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References

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Further Reading

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Keywords

Donath-Landsteiner hemolytic anemia, Donath-Landsteiner syndrome, paroxysmal cold hemoglobinuria, PCH, cryopathic hemolytic syndrome, cold-induced immune hemolytic anemia, autoimmune hemolytic anemia, AIHA, intravascular hemolysis, anemia, biphasic hemolysin, cold hemolytic syndrome, hemolytic anemia, cold agglutinin disease, hepatosplenomegaly, Raynaud phenomenon, cold urticaria, cyanosis, measles, mumps, Epstein-Barr virus, EBV, cytomegalovirus, CMV, varicella zoster virus, adenovirus, coxsackie A9, parvovirus, influenza A, Mycoplasma pneumoniae, Haemophilus influenza, Klebsiella pneumoniae, Escherichia coli, treatment, diagnosis

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Contributor Information and Disclosures

Author

M Monica Gramatges, MD, Fellow in Pediatric Hematology/Oncology, Stanford University
M Monica Gramatges, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Coauthor(s)

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 Academy of Pediatrics, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA, Senior Vice President, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
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.

 
 
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