Word Key

Achlorhydria • Lack of hydrochloric acid in the gastric contents

Intramedullary hemolysis • Premature hemolysis of red cell precursors in the bone marrow

Myelin • Fatty substance around a nerve

Paranoia • Mental conditions characterized by systematic delusions

Spasticity • Involuntary muscular contractions Paresthesias • Abnormal tingling or prickling sensation

References

1. Doig K. Anemias caused by defect of DNA metabolism. In: Rodak B, ed. Hematology: Clinical Principles and Applications, 2nd ed. Philadelphia: WB Saunders, 2002;229.

2. Lotspeich-Steininger CA. Anemias of abnormal nuclear development. In: Steine-Martin EA, Lotspeich-Steininger CA, Koepke CA, eds. Clinical Hematology: Principles, Procedures and Correlations, 2nd ed. Philadelphia: Lippincott, 1998; 156.

3. Morrison HI, Schaubel D, Desmeules M, et al. Serum folate and the risk of fatal coronary heart disease. JAMA 275:1893-1896,1996.

4. Ishtiaq O, Baqai HZ, Anwer F. Patterns of pancytopenia in a general medical ward and a proposed diagnostic approach. J Ayub Med Coll Abbottabod 16:8-13, 2004.

5. Carmel R. Cobalamin, the stomach and aging. Am J Clin Nutr 66:750-759, 1997.

6. Taghizadeh M. Megaloblastic anemias. In: Harmening D, ed. Clinical Hematology and Fundamentals of Hemostasis, 4th ed. Philadelphia: FA Davis, 2002; 118.

7. Kozyraki R, Kristiansen M, Silahtaroglu A, et al. The human intrinsic factor, vitamin B12 receptor, cubilin: Molecular characterization and chromosomal mapping of the gene to 10 p within the autosomal recessive megaloblastic anemia (MCA 1) region. Blood 91: 3593-3600, 1998.

8. Tejiani S. Pernicious anemia: Vitamin B12 to the rescue. ADVANCE for Medical Laboratory Professionals 10: 16-18, 1998.

9. Ingram CF, Fleming AF, Patel M, et al. The value of intrinsic factor antibody test in diagnosing pernicious anemia. Cent Afr J Med 44:178-181, 1998.

10. Nyhols E, Turpin P, Swain D, et al. Oral vitamin B12 can change our practice. Postgrad Med J 79:218-220, 2003.

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Copyright ©2007 by^F: A. Davis Als

Normochromic Anemias: Biochemical and Membrane Disorders and Miscellaneous Red Cell Disorders

Betty Ciesla

The Role of the Spleen in Red Cell Membrane Disorders

Hereditary Spherocytosis

The Genetics and Pathophysiology of Hereditary

Spherocytosis Clinical Presentation in Hereditary Spherocytosis Laboratory Diagnosis of Hereditary Spherocytosis Treatment and Management of Hereditary Spherocytosis

Hereditary Elliptocytosis

Common Hereditary Elliptocytosis Southeast Asian Ovalocytosis Spherocytic Hereditary Elliptocytosis Hereditary Pyropoikilocytosis

Hereditary Stomatocytosis and Hereditary Xerocytosis

Glucose-6-Phosphate Dehydrogenase Deficiency

The Genetics of Glucose-6-Phosphate Dehydroge-nase Deficiency

Clinical Manifestations of Glucose-6-Phosphate Dehydrogenase Deficiency

Diagnosis of Glucose-6-Phosphate Dehydrogenase Deficiency

Pyruvate Kinase Deficiency

Miscellaneous Red Cell Disorders

Aplastic Anemia Fanconi's Anemia Diamond-Blackfan Anemia Paroxysmal Nocturnal Hemoglobinuria Cold Agglutinin Syndrome Paroxysmal Cold Hemoglobinuria

Objectives

After completing this chapter, the student will be able to:

1. Review the functions of the spleen as they relate to red cell membrane integrity.

2. Identify the red cell membrane defect in hereditary spherocytosis.

3. Describe the clinical findings and laboratory data in patients with hereditary spherocytosis.

4. Describe the relevant red cell morphology in patients with hereditary spherocytosis.

5. Describe the osmotic fragility test and its clinical usefulness.

6. Identify the red cell membrane defects in hereditary stomatocytosis, hereditary elliptocy-tosis, and hereditary pyropoikilocytosis.

7. Compare and contrast the clinical and peripheral smear findings from hereditary stomacyto-sis, hereditary elliptocytosis, and hereditary pyropoikilocytosis.

8. Define the pathophysiology of the red cell biochemical disorders.

9. Describe the mutations and ethnic distinctions in glucose-6-phosphate dehydrogenase deficiency.

10. Describe Heinz bodies with respect to their appearance in supravital and Wright stain.

11. Define the defect in the rare membrane disorders of hereditary xerocytosis and Southeast Asian ovalocytosis.

12. Discuss the characteristics of aplastic anemia, paroxysmal nocturnal hemoglobinuria, paroxysmal cold hemoglobinuria, Fanconi's anemia, and Diamond-Blackfan syndrome.

98 Part II • Red Cell Disorders

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