eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Hemolytic Anemia: Treatment & Medication

Author: Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Contributor Information and Disclosures

Updated: Apr 27, 2009

Treatment

Medical Care

More than 200 types of hemolytic anemia exist, and each type requires specific treatment. Therefore, only the aspects of medical care relevant to most cases of hemolytic anemia are discussed here.

  • Transfusion therapy
    • Avoid transfusions unless absolutely necessary, but they may be essential for patients with angina or severely compromised cardiopulmonary status.
    • Administer packed red blood cells slowly to avoid cardiac stress.
    • In autoimmune hemolytic anemia (AIHA), type matching and cross-matching may be difficult. Use the least incompatible blood if transfusions are indicated. The risk of acute hemolysis of transfused blood is high, but the degree of the hemolysis is dependent on the rate of infusion. Slowly transfuse by administering half units of packed red blood cells to prevent rapid destruction of transfused blood.
    • Iron overload from repeated transfusions for chronic anemia (eg, thalassemia or sickle cell disorder) can be treated with chelation therapy. A recent systematic review compared the oral iron chelator deferasirox with the oral chelator deferiprone and the traditional parenteral agent, deferoxamine.10
  • Discontinuing medications
    • Discontinue penicillin and other agents that can cause immune hemolysis and oxidant medication such as sulfa drugs (see Diet).
    • Medications that can cause immune hemolysis include the following (see References for more complete lists):
      • Penicillin
      • Cephalothin
      • Ampicillin
      • Methicillin
      • Quinine
      • Quinidine
  • Administer folic acid, because active hemolysis may consume folate and cause megaloblastosis.
  • Corticosteroids are indicated in autoimmune hemolytic anemia.
  • Intravenous immunoglobulin G (IVIG) has been used for patients with AIHA, but only a few patients have had treatment responses, and the response has been transient.
  • Iron therapy
    • This is indicated for patients with severe intravascular hemolysis in which persistent hemoglobinuria has caused substantial iron loss.
    • Before iron is administered, document the iron deficiency by serum iron studies and, possibly, by assessing iron stores in bone marrow aspirates.
    • Because iron stores increase in hemolysis, iron administration is generally contraindicated in hemolytic disorders, particularly those that require chronic transfusion support.

Surgical Care

  • Splenectomy may be the first choice of treatment in some types of hemolytic anemia, such as hereditary spherocytosis.
    • In other cases, such as in AIHA, splenectomy is recommended when other measures have failed.
    • Splenectomy is usually not recommended in hemolytic disorders such as cold agglutinin hemolytic anemia.
    • Immunize against infections with encapsulated organisms, such as Haemophilus influenzae and Streptococcus pneumoniae, as far in advance of the procedure as possible.

Consultations

  • A hematology consultation helps in selecting appropriate diagnostic approaches and laboratory tests and assists in planning and monitoring therapy. Perform tests to identify hemolysis in an experienced laboratory that is selected by a hematologist.
  • Consult a general surgeon if considering splenectomy.

Diet

  • Fava beans can cause severe hemolysis in certain populations with the Mediterranean G6PD isoenzyme variant. These patients should avoid eating dishes with fava beans.
  • Medications and chemicals that should be avoided in G6PD deficiency include the following (see References for more complete lists):
    • Acetanilid
    • Furazolidone
    • Isobutyl nitrite
    • Nalidixic acid
    • Naphthalene
    • Niridazole

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications in patients with hemolytic anemia.

Vitamins

Vitamins are essential for normal DNA synthesis and the formation of a number of coenzymes in many metabolic systems.


Folic acid (Folvite)

Cofactor for enzymes involved in production of red blood cells. Replenishes depleted folate stores consumed during chronic hemolysis.

Adult

In patients with folic acid deficiency: 50 mcg/d PO/IV for 2 d, followed by 2 mg PO twice a wk or 0.5-1 mg/d

In patients with chronic hemolysis:
Administer folic acid indefinitely.

Pediatric

<12 years: Not established

>12 years: 1 mg PO/IM/SC qd

Increase in seizure frequency and a decrease in subtherapeutic levels of phenytoin reported when used concurrently

Documented hypersensitivity; folic acid administration may cause neuropathy in patients with latent or overt pernicious anemia if these patients are not receiving vitamin B-12.

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Benzyl alcohol is a preservative in some products and is associated with fatal gasping syndrome in premature infants; resistance to treatment may occur in patients with alcoholism and deficiencies of other vitamins.

Corticosteroids

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

Glucocorticoids, such as prednisone, are usually the first line of treatment in autoimmune hemolytic anemia (AIHA). Consult a hematologist to individualize therapy and determine whether other forms of therapy are indicated in the treatment of AIHA. Taper glucocorticoids very gradually to avoid a relapse of hemolysis.


Prednisone (Deltasone, Orasone, Sterapred)

Inhibits phagocytosis of antibody-covered red blood cells. Indicated in some hemolytic disorders such as AIHA.

Adult

1-1.5 mg/kg/d PO maintained for as many as 3-4 wk, or until response seen; if no response, consider other form of treatment

Pediatric

Individualize the pediatric dose because corticosteroids may affect growth; consult a pediatric hematologist to establish the dose of corticosteroids and determine whether other forms of therapy are needed.

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

More on Hemolytic Anemia

Overview: Hemolytic Anemia
Differential Diagnoses & Workup: Hemolytic Anemia
Treatment & Medication: Hemolytic Anemia
Follow-up: Hemolytic Anemia
Multimedia: Hemolytic Anemia
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Chiao EY, Engels EA, Kramer JR, et al. Risk of immune thrombocytopenic purpura and autoimmune hemolytic anemia among 120 908 US veterans with hepatitis C virus infection. Arch Intern Med. Feb 23 2009;169(4):357-63. [Medline].

  2. Hashimoto C. Autoimmune hemolytic anemia. Clin Rev Allergy Immunol. Fall 1998;16(3):285-95. [Medline].

  3. Packman CH. Hemolytic anemia due to warm autoantibodies. Blood Rev. Jan 2008;22(1):17-31. [Medline].

  4. Mayer B, Yurek S, Kiesewetter H, Salama A. Mixed-type autoimmune hemolytic anemia: differential diagnosis and a critical review of reported cases. Transfusion. Oct 2008;48(10):2229-34. [Medline].

  5. Sanz J, Arriaga F, Montesinos P, et al. Autoimmune hemolytic anemia following allogeneic hematopoietic stem cell transplantation in adult patients. Bone Marrow Transplant. May 2007;39(9):555-61. [Medline].

  6. George JN. The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: overview of pathogenesis (Experience of The Oklahoma TTP-HUS Registry, 1989-2007). Kidney Int Suppl. Feb 2009;S8-S10. [Medline].

  7. Dhingra KK, Jain D, Mandal S, et al. Evans syndrome: a study of six cases with review of literature. Hematology. Dec 2008;13(6):356-60. [Medline].

  8. Garratty G. Immune hemolytic anemia associated with negative routine serology. Semin Hematol. Jul 2005;42(3):156-64. [Medline].

  9. Kamesaki T, Oyamada T, Omine M, Ozawa K, Kajii E. Cut-off value of red-blood-cell-bound IgG for the diagnosis of Coombs-negative autoimmune hemolytic anemia. Am J Hematol. Feb 2009;84(2):98-101. [Medline]. [Full Text].

  10. [Best Evidence] McLeod C, Fleeman N, Kirkham J, et al. Deferasirox for the treatment of iron overload associated with regular blood transfusions (transfusional haemosiderosis) in patients suffering with chronic anaemia: a systematic review and economic evaluation. Health Technol Assess. Jan 2009;13(1):iii-iv, ix-xi, 1-121. [Medline]. [Full Text].

  11. Beutler E. G6PD: population genetics and clinical manifestations. Blood Rev. Mar 1996;10(1):45-52. [Medline].

  12. Beutler E. Hemolytic anemia due to infections with microorganisms. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:674-6.

  13. Beutler E. Hemolytic anemia due to chemical and physical agents. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:670-3.

  14. Erslev AJ. Traumatic cardiac hemolytic anemia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:663-4.

  15. Glader BE. Hemolytic anemia in children. Clin Lab Med. Mar 1999;19(1):87-111, vi. [Medline].

  16. Hamilton JW, Jones FG, McMullin MF. Glucose-6-phosphate dehydrogenase Guadalajara--a case of chronic non-spherocytic haemolytic anaemia responding to splenectomy and the role of splenectomy in this disorder. Hematology. Aug 2004;9(4):307-9. [Medline].

  17. Ideguchi H, Ishikawa A, Futata Y, et al. A comprehensive scheme for the systematic investigation of hemolytic anemia. Ann Clin Lab Sci. Sep-Oct 1994;24(5):412-21. [Medline].

  18. Jubinsky PT, Rashid N. Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. Pediatr Blood Cancer. Sep 2005;45(3):347-50. [Medline].

  19. Kong JT, Schmiesing C. Concealed mothball abuse prior to anesthesia: mothballs, inhalants, and their management. Acta Anaesthesiol Scand. Jan 2005;49(1):113-6. [Medline].

  20. Lane DR, Youse JS. Coombs-positive hemolytic anemia secondary to brown recluse spider bite: a review of the literature and discussion of treatment. Cutis. Dec 2004;74(6):341-7. [Medline].

  21. Martinez J. Microangiopathic hemolytic anemia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:669.

  22. Packman CH, Leddy JP. Drug-related immune hemolytic anemia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:691-6.

  23. Packman CH, Leddy JP. Acquired hemolytic anemia due to warm-reacting autoantibodies. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:667-84.

  24. Packman CH, Leddy JP. Cryopathic hemolytic syndromes. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:685-90.

  25. Palek J, Jarolim P. Red cell membrane disorders. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 2nd ed. New York, NY: Churchill Livingstone; 1995:667-709.

  26. Palek J, Jarolim P. Hereditary spherocytosis, elliptocytosis, and related disorders. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:557-63.

  27. Petz LD. Drug-induced autoimmune hemolytic anemia. Transfus Med Rev. Oct 1993;7(4):242-54. [Medline].

  28. Ramanathan S, Koutts J, Hertzberg MS. Two cases of refractory warm autoimmune hemolytic anemia treated with rituximab. Am J Hematol. Feb 2005;78(2):123-6. [Medline]. [Full Text].

  29. Schrier S. Extrinsic nonimmune hemolytic anemias. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 2nd ed. New York, NY: Churchill Livingstone; 1995:729-36.

  30. Schwartz RS, Silverstein LE, Berkman EM. Autoimmune hemolytic anemias. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 2nd ed. New York, NY: Churchill Livingstone; 1995:710-28.

  31. Shah A. Acquired hemolytic anemia. Indian J Med Sci. Dec 2004;58(12):533-6. [Medline]. [Full Text].

  32. Sharma A, Khanduri U, Kumar P. Hematology morphology forum. Paroxysmal cold haemoglobinuria. Hematology. Aug 2004;9(4):315-6. [Medline].

  33. Tabbara IA. Hemolytic anemias. Diagnosis and management. Med Clin North Am. May 1992;76(3):649-68. [Medline].

  34. Tang S, Cheng IK, Leung VK, et al. Successful treatment of hepatitis C after kidney transplantation with combined interferon alpha-2b and ribavirin. J Hepatol. Nov 2003;39(5):875-8. [Medline].

  35. Toye AM, Ghosh S, Young MT, et al. Protein-4.2 association with band 3 (AE1, SLCA4) in Xenopus oocytes: effects of three natural protein-4.2 mutations associated with hemolytic anemia. Blood. May 15 2005;105(10):4088-95. [Medline]. [Full Text].

  36. Zamvar V, McClean P, Odeka E, Richards M, Davison S. Hepatitis E virus infection with nonimmune hemolytic anemia. J Pediatr Gastroenterol Nutr. Feb 2005;40(2):223-5. [Medline].

[ CLOSE WINDOW ]

Further Reading

Related eMedicine Topics

Clinical Trials

National Guidelines Clearinghouse

[ CLOSE WINDOW ]

Keywords

hemolytic anemia, autoimmune hemolytic anemia, anemia, hemolysis, microangiopathic anemia, premature erythrocyte destruction, hereditary hemoglobin abnormalities, glucose-6-phosphate dehydrogenase deficiency, G6PD deficiency, G-6-PD deficiency, hereditary spherocytosis, sickle cell anemia, sickle cell trait, sickle cell disease, AIHA, disseminated intravascular coagulation, DIC, hemolytic uremic syndrome, HUS, hemolytic-uremic syndrome, TTP, thrombotic thrombocytopenic purpura, defective prosthetic cardiac valves, parvovirus B19 infection

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Medical Editor

Rodger L Bick, MD, PhD, FACP, Clinical Professor of Medicine, University of Texas Southwestern Medical Center; Director, Dallas and Pacific Thrombosis Hemostasis and Vascular Medicine Clinical Center
Rodger L Bick, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Blood Banks, American Cancer Society, American College of Angiology, American College of Physicians, American Geriatrics Society, American Heart Association, American Medical Association, American Society for Clinical Pathology, American Society of Hematology, Association of Clinical Scientists, California Medical Association, California Thoracic Society, International College of Angiology, International Society of Hematology, International Society on Thrombosis and Haemostasis, New York Academy of Sciences, and Southwest Oncology Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Director of the Hoxworth Blood Center, Professor, Departments of Internal Medicine and Pathology, University of Cincinnati Medical Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.