eMedicine Specialties > Pediatrics: General Medicine > Hematology

Donath-Landsteiner Hemolytic Anemia: Treatment & Medication

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

Treatment

Medical Care

Treatment of Donath-Landsteiner hemolytic anemia (DLHA) depends on the severity of the disease and the presence of an underlying cause. In children, Donath-Landsteiner hemolytic anemia is usually transient; therefore, management is by observation only. However, the anemia may be so severe as to be life-threatening, and supportive care with transfusions is often warranted.14 Guidelines for transfusions in neonates and older children have been established.15

  • Although exposure to cold is not always associated with disease presentation, supportive care recommendations include avoidance of extreme cold exposure based on understanding of disease pathophysiology (see above). Supportive care may also include hydration if hemoglobinuria is present and judicious use of RBC transfusions. 
  • RBC transfusion is indicated when anemia is severe or symptomatic.
    • Perform compatibility testing using the techniques described above to minimize the interference caused by the autoantibody (see Workup).
    • Although P antigen-negative RBCs are most efficacious, these units are extremely rare (most banked blood units are P antigen positive). Blood transfusion may be safely accomplished with P antigen-positive units in most cases, resulting in the expected hemoglobin increases based on the amount administered. 
    • Use blood warmers if possible to perform the RBC transfusion at 37°C. 
  • Viral infections are self-limited. Treat syphilis and mycoplasma infections with appropriate antibiotics. Hemolysis resolves with treatment of the infection. 
  • Corticosteroid therapy may be indicated in patients with Donath-Landsteiner hemolytic anemia and severe anemia. 
  • Splenectomy has no role in treatment of Donath-Landsteiner hemolytic anemia because the hemolysis is intravascular. 
  • Plasmapheresis has been successfully used in extremely severe cases that are refractory to transfusion16  
  • Rituximab has been used to treat refractory autoimmune hemolytic anemia, including cold agglutinin disease. Although treatment of Donath-Landsteiner hemolytic anemia beyond the use of steroids is rarely required, rare refractory cases (particularly in adults) have been described in which rituximab was used successfully.17

Consultations

  • Consult a hematologist and/or blood bank specialist to aid in the diagnosis and treatment of Donath-Landsteiner hemolytic anemia.

Medication

Prednisone may be effective in controlling hemolysis. However, evidence for effectiveness in Donath-Landsteiner hemolytic anemia is anecdotal.

Glucocorticoids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.


Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Adult

60 mg/d PO qd or divided bid/tid; taper over 2 wk as symptoms resolve

Pediatric

2 mg/kg/d PO divided bid/tid; taper over 2 wk as symptoms resolve

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

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

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

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

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

  6. 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].

  7. 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].

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

  9. 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].

  10. 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].

  11. 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].

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

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

  14. Gertz MA. Management of cold haemolytic syndrome. Br J Haematol. Aug 2007;138(4):422-9. [Medline].

  15. [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].

  16. 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].

  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. 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].

  19. 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].

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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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