Anemia - Krafts Flashcards
Name two types of hemolytic anemias:
Hereditary Spherocytosis
Autoimmune Hemolytic Anemia
What anemias have weird shaped RBCs?
Hemolytic Anemias
Sickle Cell Anemias
Define Hemoglobinopathies
Change in the quality or structure of the hemoglobin
Define Poikiocytosis
Weird Shaped RBCs
What happens in Sickle Cells?
Hemolysis
Vaso-occlusion
What is the most important hemoglobinopathy?
Sickle Cell Anemia
What is the usual cause of hemoglobinopathies?
A single amino acid (Valine to Glutamate) point mutation in the B-Globin chain
What is the best way to test for hemoglobinopathies?
Hemoglobin Electrophoresis
What are the genes for Sickle-Cell Trait?
HbA - HbS
What are the genes for Sickle-Cell Anemia?
HbS - HbS
Sickle Cell Anemia Characteristics
Blacks (8% are heterozygous)
Variable severity
Increased infections due to autosplenectomy
What causes an autosplenectomy?
Spleen shrinks down due to infarctions healing and causing fibrosis. Spleen is needed to kill encapsulated organisms (pneumococcal and H. flu)
Morphology of Sickle Cell Anemia (Post-Splenectomy
Nucleated RBCs Target RBCs Howell-Jolly Bodies Pappenheimer Bodies Increased Platelet Count
Target RBCs
Too much membrane or not enough volume
Pappenheimer Bodies
Aggregates of iron within the cell, usually removed by macrophages
What are the triggers for sickle cell anemia that need to be prevented?
Infection, Fever, Dehydration, Hypoxemia (keep patient from de-oxygenating)
What vaccinations should be given to Sickle Cell Anemia patients when the spleen is autosplenectomized?
Encapsulated bugs
Streptococcus Pneumoniae
Hemophilus Influenzae
What are the other treatments for Sickle Cell Anemia?
Blood Transfusions
Bone Marrow Transplantations (in children)
Thalassemia
Hypochromic, Microcytic Anemia Can't make enough alpha or beta hemoglobin chains See Target Cells Basophilic Stippling Medullary Expansion
Normal Globin Genes
4 alpha-chain genes
2 beta-chain genes
Alpha Thalassemia Gene Defect
Deletion of one or more Alpha-chain gene(s)
Beta Thalassemia Gene Defect
Defective Beta-chain gene(s)
Cause of B-Thalassemia
Defective transcription, translation, or processing of mRNA of B-chain gene
Severity of B-Thalassemia
B gene = normal gene
B+ gene = produces some B-chains
B0 gene = produces no B-chains