Thalassemias and Related Disorders: Quantitative Disorders of Hemoglobin Synthesis Caterina Borgna-Pignatti Renzo Galanello* Dedication: We wish to dedicate this chapter to Rino Vullo and Antonio Cao who passed away on…
Hemoglobins with Altered Oxygen Affinity, Unstable Hemoglobins, M-Hemoglobins, and Dyshemoglobinemias Martin H. Steinberg More than 1,100 mutations affecting the globin subunits of hemoglobin have been described (http://globin.cse.psu.edu/), and among these,…
Sickle Cell Anemia and Other Sickling Syndromes Jane S. Hankins Winfred C. Wang This chapter discusses hemoglobin (Hb) variants that cause alterations in erythrocyte morphology and rheology. Hb S, or…
Acquired Nonimmune Hemolytic Disorders Robert T. Means, Jr. Bertil Glader Hemolysis occurs when red blood cells (RBCs) are exposed to a variety of infectious agents, chemicals, or physical stresses. In…
Paroxysmal Nocturnal Hemoglobinuria Charles J. Parker Russell E. Ware INTRODUCTION Definition Although usually classified as a hemolytic anemia, paroxysmal nocturnal hemoglobinuria (PNH) is a disease of the hematopoietic stem cell….
Hemolytic Disease of the Fetus and Newborn Charles T. Quinn Anne F. Eder Catherine S. Manno Hemolytic disease of the fetus and newborn (HDFN) results from the destruction of red…
Autoimmune Hemolytic Anemia Richard C. Friedberg Vandita P. Johari CLASSIFICATION In autoimmune hemolytic anemia (AIHA), pathologic antibodies (autoantibodies) attach to and lead to the destruction (hemolysis) of endogenous erythrocytes (red…
Hereditary Hemolytic Anemias Due to Red Blood Cell Enzyme Disorders Bertil Glader OVERVIEW OF ERYTHROCYTE METABOLISM Mature red blood cells (RBCs) are anucleate (thereby incapable of cell division), devoid of…
Hereditary Spherocytosis, Hereditary Elliptocytosis, and Other Disorders Associated with Abnormalities of the Erythrocyte Membrane Patrick G. Gallagher Bertil Glader This chapter focuses on hemolytic disorders and abnormalities of red blood…
Porphyrias Sylvia S. Bottomley The porphyrias are diverse disorders that arise from various inherited enzyme defects in the heme biosynthesis pathway (Fig. 26.1). The first authentic cases of porphyria were…
