Hemolytic Anemia Clinical Presentation

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

History

Symptoms of hemolytic anemia are diverse and are due to anemia, the extent of compensation, previous treatment, and the underlying disorder. Patients with minimal or long-standing hemolytic anemia may be asymptomatic, and hemolysis is often found incidentally during routine laboratory testing.

In intravascular hemolysis, iron deficiency due to chronic hemoglobinuria can exacerbate anemia and weakness.

Tachycardia, dyspnea, angina, and weakness occur in patients with severe anemia. Cardiac function is sensitive to anoxia.

Persistent hemolysis may result in the development of bilirubin gallstones. These patients may present with abdominal pain.

Bronze skin color and diabetes occur in hematosiderosis. Iron overload may occur in patients who have received multiple transfusions or those who have been erroneously administered iron therapy.

Dark urine may be due to hemoglobinuria.

In addition to hemolysis, patients with thrombotic thrombocytopenic purpura (TTP) may experience fever, neurologic signs, renal failure, and thrombocytopenia.

Leg ulcers may develop in patients with sickle cell anemia and other hemolytic disorders, as a result of decreased RBC deformability and endothelial changes.

Patients may report recent use of medications that can cause immune hemolysis; these include penicillin, quinine, quinidine, and L-dopa.

In patients with G-6-PD deficiency, oxidant drugs and stress from infections can trigger hemolysis. Fava beans can induce hemolysis in susceptible individuals with the Mediterranean variant of G-6-PD deficiency.

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Physical Examination

The physical examination in an individual with hemolytic anemia can reveal signs of anemia, complications of hemolysis, and evidence of an underlying disease. General pallor and pale conjunctivae and fingernails indicate anemia but are not specific for hemolytic anemias. Tachycardia, tachypnea, and hypotension due to anoxia and decreased vascular volume can occur in severe anemias but are not specific for hemolytic anemias.

Jaundice may occur because of a modest increase in indirect bilirubin in hemolysis. The rise is not specific for hemolytic disorders and may occur in liver disease and biliary obstruction. Bilirubin levels are rarely greater than 3 mg/dL in hemolysis, unless complicated by hepatic disease or cholelithiasis.

Splenomegaly occurs in hereditary spherocytosis and other hemolytic anemias, but it is not present in all hemolytic disorders. For example, splenomegaly usually is not present in G-6-PD deficiency. The presence of splenomegaly could suggest an underlying disorder such as chronic lymphocytic leukemia (CLL), some lymphomas, or systemic lupus erythematosus (SLE). Butterfly malar rash and arthritis also suggest SLE. Lymphadenopathy along with splenomegaly is consistent with CLL. Splenomegaly sometimes is not evident on physical examination, and ultrasonic imaging or CT scanning may be necessary to define spleen size. When evaluating spleen size, it is important to avoid unnecessary pressure in order to avoid splenic rupture.

Leg ulcers may be present.

Right upper abdominal quadrant tenderness may indicate cholelithiasis (bilirubin gallstones) and gallbladder disease.

Tachycardia and dyspnea may be evident when the onset of hemolysis is abrupt and the anemia is severe. Angina and heart failure symptoms can occur in patients with underlying cardiovascular disease.

In patients with chronic hemolytic anemia, increased folate consumption may lead to folate deficiency. Clinical manifestations may include patchy hyperpigmentation, sore tongue, and GI symptoms.

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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.

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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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