Hemolytic Anemia Treatment & Management

  • Author: Paul Schick, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Aug 8, 2011
 

Approach Considerations

There are numerous types of hemolytic anemia, and treatment may differ depending on the type of hemolysis.[2, 3, 4, 5, 28, 29] Only the general care of hemolytic anemias and the management of the most commonly encountered hemolytic anemias are discussed.

Folic acid, corticosteroids, and IVIG

Prophylactic folic acid is indicated because active hemolysis can 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 responded to this treatment, and the responses have been transient.

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Transfusion Therapy

One should avoid transfusions unless absolutely necessary. However, transfusions may be essential for patients with angina or a severely compromised cardiopulmonary status. It is best to administer packed red blood cells slowly to avoid cardiac stress.

In autoimmune hemolytic anemia (AIHA), typing and cross-matching may be difficult. One should use the least incompatible blood if transfusions are indicated. The risk of destruction of transfused blood is high, but the degree of the hemolysis depends on the rate of infusion. Therefore, one should slowly transfuse half units of packed red blood cells to prevent rapid destruction of transfused blood.

Iron overload due to multiple transfusions for chronic anemia (eg, thalassemia or sickle cell disorder) can be treated with chelation therapy. A systematic review that compared the oral iron chelator deferasirox with the oral chelator deferiprone and the traditional parenteral agent deferoxamine found little clinical difference between the 3 chelation agents in terms of removing iron from the blood and liver.[30]

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Discontinuing Medications

Penicillin and other agents that can cause immune hemolysis should be discontinued in patients who develop hemolysis. This is a partial list of medications that can cause immune hemolysis: penicillin, cephalothin, ampicillin, methicillin, quinine, quinidine.

One should discontinue oxidant medications such as sulfa drugs in patients with G-6-PD deficiency or those who have unstable hemoglobins. This is a partial list of medications and chemicals that should be avoided in G-6-PD deficiency: acetanilid, furazolidone, isobutyl nitrite, nalidixic acid, naphthalene, niridazole.

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Iron Therapy

Iron therapy is contraindicated in most cases of hemolytic anemia. The reason is that iron released from RBCs in most hemolytic anemias is reused and iron stores are not reduced.

However, iron therapy is indicated for patients with severe or intravascular hemolysis in which persistent hemoglobinuria has caused substantial iron loss. Before starting iron therapy, one should document iron deficiency by serum iron studies and, possibly, by assessing iron stores in bone marrow aspirates.

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Splenectomy

Splenectomy may be the first choice of treatment in some types of hemolytic anemia, such as hereditary spherocytosis.[31] 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 in which hemolysis is intravascular.

Overwhelming postsplenectomy sepsis is a rare but a potentially fatal event, especially during the first 2 years after splenectomy. One should immunize against infections with encapsulated organisms, such as Haemophilus influenzae and Streptococcus pneumoniae, in advance of the procedure. Immunization can be performed post splenectomy.

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Deterrence/Prevention of Hemolytic Anemia

This is a partial list of medications and chemicals that should be avoided in G-6-PD deficiency: acetanilid, furazolidone, isobutyl nitrite, nalidixic acid, naphthalene, niridazole, sulfa drugs.

The G-6-PD Deficiency Association has a more comprehensive online list of medications that people with G-6-PD deficiency should avoid.

Fava beans can cause severe hemolysis in certain populations with the Mediterranean G-6-PD isoenzyme variant. These patients should avoid eating dishes with fava beans.

Patients should know to avoid medications that caused them to have immune hemolysis. This is a partial list of medications that can cause immune hemolysis: penicillin, cephalothin, ampicillin, methicillin, quinine, quinidine.

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Consultations

A hematology consultation would be helpful in selecting appropriate diagnostic approaches and laboratory tests and in planning and monitoring therapy.

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Long-Term Monitoring

One should monitor the hemoglobin level, reticulocyte count, indirect bilirubin value, LDH level, and haptoglobin value in patients with hemolytic anemia to determine the response to therapy. Urine hemoglobin and hemosiderin should be monitored to evaluate recovery in patients with severe or intravascular hemolysis.

Folic acid should be recommended for patients with ongoing hemolysis.

Administer oral iron to patients who have become iron deficient due to intravascular hemolysis.

One should taper corticosteroids. Occasionally, patients may have to continue low-dose steroids.

Avoid transfusions unless there is evidence of angina, cardiopulmonary decompensation, or other severe organ impairment.

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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Paul Schick, MD  Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

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.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC  Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of 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 Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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Polychromasia.
Spherocytes. One arrow points to a spherocyte; the other, to a normal RBC with central pallor.
Schistocytes (thrombotic thrombocytopenic purpura).
Peripheral blood smear with sickled cells at 1000X magnification. Image courtesy of Ulrich Woermann, MD.
Supra vital stain in hemoglobin H disease that reveals Heinz bodies (golf ball appearance).
 
 
 
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