Certified Pediatric Hematology Oncology Nurse (CPHON) Practice Exam 2025 - Free CPHON Practice Questions and Study Guide

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Thalassemia is defined as:

A group of inherited disorders affecting RBCs

Thalassemia is a group of inherited disorders that affect the production of hemoglobin, the protein in red blood cells (RBCs) responsible for oxygen transport. In thalassemia, there is a reduction in the production of specific globin chains that form the hemoglobin molecule, leading to anemia and various complications.

A distinguishing feature of thalassemia is its classification into different types, namely alpha and beta thalassemia, based on which globin chain is affected. These disorders are typically inherited in an autosomal recessive manner. The impact on the red blood cells includes not only reduced hemoglobin production but also structural abnormalities and increased destruction of red blood cells, contributing to the overall clinical picture.

The other definitions presented do not fully encapsulate what thalassemia represents. For example, referring solely to thalassemia as a single-gene disorder does not consider the varied types and complexities involved. Additionally, describing it as a normal variant of hemoglobin is misleading, given that thalassemia results in significant health issues and not merely a benign variation. Lastly, while thalassemia can lead to hemolysis, it is not classified exclusively as a hemolytic condition, as it also involves

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A single-gene disorder only

A normal variant of hemoglobin

A hemolytic condition only

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