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Unstable hemoglobin disease is rare and may be caused by any 1 of a large number of hemoglobin variants. They are inherited as an autosomal dominant trait. The severity of the disease varies according to the hemoglobin variant; there may be no clinical symptoms or the disease may produce a mild, moderate, or severe hemolytic anemia.
The stained peripheral blood smear shows anisocytosis, poikilocytosis, basophilic stippling, polychromasia and, sometimes, hypochromia. The reticulocyte count may be increased. Splenomegaly and Heinz bodies may also be present.
Work-up of congenital hemolytic anemias
An abnormal or unstable result is indicative of a hemoglobin variant present. Other confirmatory tests should be performed to identify the hemoglobinopathy (HBELC / Hemoglobin Electrophoresis Cascade, Blood).
False positives will be obtained in blood specimens containing >5% fetal hemoglobin or in specimens >1 week old.
Only orderable as part of a profile or as a reflex. For more information see HAEVP / Hemolytic Anemia Evaluation; or HBELC / Hemoglobin Electrophoresis Cascade, Blood; or THEVP / Thalassemia and Hemoglobinopathy Evaluation; or REVE / Erthrocytosis Evaluation; or MEVP / Methemoglobinemia Evaluation.
Normal (reported as normal [stable] or abnormal [unstable])
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