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Chapter 6
Hemolytic anemias
Overview
The hemolytic anemias are a diverse group of disorders characterized by accelerated red cell destruction, typically with an unimpaired ability of the bone marrow to respond with increased erythrocyte production. Anemia may not be evident if the rate of hemolysis is modest and the bone marrow is able to compensate for the decreased red cell life span. However, any stress to the hematopoietic system leading to decreased red blood cell (RBC) production can lead to marked anemia.
The hemolytic anemias can be classified in several ways. They can be inherited (eg, sickle cell disease, hereditary spherocytosis) or acquired (eg, autoimmune,
Congenital hemolytic anemia
Organization and expression of globin genes Hemoglobin structure Hemoglobin function Thalassemias ß Thalassemias Molecular basis Pathophysiology Clinical features Laboratory findings Management of the ß thalassemias  ThalassemiasMolecular basis Pathophysiology Clinical features Laboratory features Clinical management of thalassemiasHemoglobinopathies Sickle syndromes Pathophysiology Laboratory features Clinical manifestations Treatment Preventive interventions Painful episodes Red blood cell transfusion Acute chest syndrome Central nervous system disease Pregnancy Modifying the disease course Hemoglobin C disease Hemoglobin D disease Hemoglobin E syndromes Unstable hemoglobinopathies Hemoglobin mutants with altered oxygen affinity Acquired hemoglobinopathies Methemoglobinemia Sulfhemoglobinemia Hereditary spherocytosis, hereditary elliptocytosis, and hereditary pyropoikilocytosis Red cell membrane protein composition and assembly Hereditary spherocytosis Pathophysiology Clinical manifestations Laboratory evaluation Treatment Hereditary elliptocytosis and hereditary pyropoikilocytosis Pathophysiology Clinical manifestations, laboratory evaluation, and treatment Other red cell membrane disorders Acanthocytosis Stomatocytosis Rh deficiency syndrome Hemolytic anemias due to red cell metabolic disorders Hemolytic anemia due to enzyme abnormalities of the glycolytic pathway Hemolytic anemia due to disorders of the hexose-monophosphate shunt Red cell enzymopathies of purine and pyrimidine metabolism Pyrimidine-5'-nucleotidase deficiency Adenosine deaminase excess
Acquired hemolytic anemia
Immune-mediated hemolytic disorders Pathophysiology Drug-induced immune hemolytic anemia Clinical manifestations and laboratory findings Treatment Transfusion in autoimmune hemolytic anemias Other therapies Paroxysmal nocturnal hemoglobinuria Pathophysiology Laboratory findings Laboratory diagnosis Clinical manifestations Treatment Prognosis Hemolytic anemia due to red cell fragmentation Heart and great vessels Microangiopathic disease Hemolytic anemia due to chemical or physical agents Hemolytic anemia due to infection Red cell membrane injury caused by bacteria Hemolytic anemias with gram-positive and gram-negative organisms Immune hemolysis associated with infections Hemolytic anemia associated with parasitic infestation of red cells   CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
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