January 19, 2025

An 18-year-old boy had a CBC performed as part of a college physical

By Med-Challenger · 1 minute read

An 18-year-old boy had a complete blood count (CBC) performed as part of a college physical examination. He was asymptomatic. CBC was remarkable for mild anemia with a hemoglobin level of 12 g/dL and mean corpuscular volume that was quite low at 65 fL. He denied any hematochezia, melena, or hematuria, and his urinalysis finding was normal. Iron levels, ferritin level, total iron-binding capacity, and transferrin saturation levels were normal.

On hemoglobin electrophoresis, the patient is found to have reduced hemoglobin A levels (2 alpha chains and 2 beta chains) and increased levels of hemoglobin A2 (2 alpha chains and 2 delta chains).

Which of the following is the appropriate management approach for this patient’s anemia?

transfusion of packed red blood cells (PRBCs)
cyclophosphamide therapy
observation
corticosteroids

The correct answer is:

observation

Educational Objective:

Manage thalassemia minor.

Key Point:

Often characterized by mild microcytic anemia, beta thalassemia minor does not generally require treatment, whereas patients with beta thalassemia major require transfusion.

Explanation:

In this case, observation is the appropriate management approach for beta thalassemia minor.

Thalassemias are genetic disorders distinguished by abnormal hemoglobin chains. In alpha thalassemia, the alpha chains are reduced or absent. In beta thalassemia major, both genes for beta hemoglobin are defective, and there is severe anemia evident in infancy and accompanied by delayed growth and other abnormalities. It is treated with lifelong transfusion, often resulting in iron overload and the need for chelation. In beta thalassemia minor, or thalassemia trait, only one gene defect is present, and patients are often asymptomatic, perhaps having mild microcytic anemia. Patients with beta thalassemia minor require no treatment.

Transfusion with PRBCs and sometimes hemopoietic stem cell transplantation are treatments for beta thalassemia major. With many transfusions, iron overload is an additional risk for patients with beta thalassemia major.

References:

Capellini MD. The Thalassemias (Chapter 162). In: Goldman L, Schafer A. Goldman-Cecil Medicine. 25th ed., 2016:1089-1095.

Sayani FA, Kwiatkowski JL. Increasing prevalence of thalassemia in America: implications for primary care. Ann Med. 2015;47:592-604.

This question appears in Med-Challenger Physician Assistant Exam Review with CME

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