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The page below is a sample from the LabCE course Alpha Thalassemia. Access the complete course and earn ASCLS P.A.C.E.-approved continuing education credits by subscribing online.

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The normal RBC count (4.84 x 1012/L) in this case, together with the decreased hemoglobin (8.4 g/dL) and MCV (59 fl) is an indicator of ineffective erythropoeisis that often points to thalassemia.

The RBC morphology shows slight hypochromic microcytosis with codocytes, schistocytes, and basophilic stippling. Schistocytes form by several mechanisms, one being the removal of RBC inclusions.

This patient's elevated bilirubin correlates with her presentation of sclera icterus; her splenomegaly is consistent with increased RBC destruction.

The Hb electrophoresis demonstrated a normal pattern, initially, but the unstable Hemoglobin H was revealed upon repeat electrophoresis with reduced incubation time. Hemoglobin H is the result of beta globin chain tetramer formation due to the insufficient supply of alpha globin chains in alpha thalassemia intermedia.

People with Hemoglobin H disease (alpha thalassemia intermedia) usually have a normal life expectancy without treatment. However, hemolysis may lead to moderate anemia that may be treated with splenectomy.