The Hematopoietic and Lymphoid Systems

Chapter 9 The Hematopoietic and Lymphoid Systems





RED BLOOD CELL DISORDERS


















17 What is the difference between anemia caused by extrinsic factors (extracorpuscular defects) and anemia caused by intrinsic factors (intracorpuscular defects)?


Because the classification of intravascular and extravascular hemolytic disorders is not entirely satisfactory, a classification based on the underlying cause of red cell destruction is proposed:







19 What are the exact molecular defects in HS?


The normal membrane cytoskeleton of RBCs is composed of several proteins, the most important of which are α and β spectrin, ankyrin, actin, and proteins known as band 4.1 and band 3 (Fig. 9-2). Together these proteins maintain the normal biconcave shape of RBCs. The mutation of ankyrin gene is the most common defect in autosomal dominant HS, and the mutations of gene encoding protein band 3 account for 20% of cases. Genes encoding other cytoskeletal proteins are less often mutated.


















34 What are three types of crisis in sickle cell anemia?










38 What are the pathologic features of β-thalassemia?





















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Jul 23, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on The Hematopoietic and Lymphoid Systems

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