Laboratory Findings in Alpha Thalassemia: Hematology

Alpha Thalassemia Major

Anemia is fatal.
Red blood cell (RBC) count is increased.
Hemoglobin (Hb) is severely decreased.
Mean corpuscular volume (MCV) is decreased.
Mean corpuscular hemoglobin concentration (MCHC) is decreased.
Red cell distribution width (RDW) is increased.
RBC morphology shows slight hypochromic microcytosis with codocytes, schizocytes, nucleated RBCs.
Reticulocytes are increased.
Hb electrophoresis demonstrates abnormal pattern on cord blood:

Hb A - absent
Hb Bart's - 80-90%
Hb Portland - 0-20%

Bone marrow demonstrates marked erythroid hyperplasia.

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