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Evaluation of lifelong or inherited hemolytic anemias
This is a consultative evaluation in which the case will be evaluated and appropriate tests performed, at an additional charge, and the results interpreted.
Eosin-5-malemide (EMA) binding (band 3) flow cytometry will be performed on all cases.
Note: RBCE / Reflexed RBC Enzymes, Blood includes: adenosine deaminase, adenylate kinase, phosphofructokinase, phosphoglycerate kinase, triosephosphate isomerase, and pyrimidine 5' nucleotidase.
Hemolytic anemia (HA) is characterized by increased red cell destruction and a decreased red cell life span. Patients usually have decreased hemoglobin concentration, hematocrit, and red blood cell count, but some can have compensated disorders, and symptoms such as reticulocytosis, pigmented gallstones, and decreased haptoglobin are factors that raise clinical suspicion. Blood smear abnormalities may include spherocytes, schistocytes, stomatocytes, polychromasia, basophilic stippling, and target cells. Osmotic fragility can be increased due to the presence of spherocytes.
HAs may be congenital or acquired. Inherited hemolytic disorders may include red cell membrane disorders, red cell enzyme defects, or abnormalities in the hemoglobin molecule in the red cell. This panel assesses for possible causes of congenital/hereditary causes of hemolytic anemia and does not evaluate for acquired causes. Therefore, the anemia should be lifelong or familial in nature. Examples of acquired HA, which should be excluded prior to ordering this panel, include: autoimmune HA, direct Coombs-positive HA, cold agglutinin disease, disseminated intravascular coagulation, and drug-induced HA.
This consultative evaluation looks for the cause of increased red cell destruction and includes testing for red cell membrane disorders, such as hereditary spherocytosis, hemoglobinopathies, and red cell enzyme abnormalities.
Definitive results and an interpretive report will be provided.
An interpretive report will be provided.
Preliminary screening tests, such as complete blood count with peripheral smear and direct Coombs test with a negative result, should be run before ordering this evaluation.
This panel is most effectively interpreted in the context of clinical information and the peripheral blood morphology. Please fill out the Hemolytic Anemia Patient Information Sheet (Supply T705) available in Special Instructions to maximize the interpretive capabilities of the panel.
This group of tests should not ordinarily be requested in patients who are likely to have immune hemolytic anemia (HA), such as that due to either warm or cold antibodies or to paroxysmal nocturnal hemoglobinurias. Coombs tests, tests for cold agglutinins, sucrose hemolysis, and Hams and Crosby tests are not part of the HA evaluation. In general, the foregoing tests should have been performed and found to be negative prior to requesting an HA evaluation. Since Wilson disease is another rare cause for acute intermittent hemolysis, testing for Wilson disease also may be appropriate prior to requesting an HA evaluation.
1. Beutler E: Glucose-6-phosphate dehydrogenase deficiency and other enzyme abnormalities. In Hematology. Fifth edition. Edited by E Beutler, MA Lichtman, BS Coller, TJ Kipps. New York, McGraw-Hill Book Company, 1995, pp 564-581
2. Hoyer JD, Hoffman DR: The thalassemia and hemoglobinopathy syndromes. In Clinical Laboratory Medicine. Second edition. Edited by KD McMlatchey. Philadelphia, Lippincott, Williams and Wilkins, 2002, pp 866-895