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
B-Thalassemia Minor
Asymoptomatic
B-Thalassemia Intermedia
Less Severe Thalassemia
B-Thalassemia Major
Severe Thalassemia
-a/aa Thalassemia
Silent
–/aa or -a/-a
Alpha-Thalassemia Trait: Asymptomatic
–/-a
Hemoglobin H Disease: Moderate/Severe Disease
–/– (No alpha chain genes)
Hydrops Fetalis: Fatal in utero
What causes anemia in a-thalassemia?
Not enough a-chains
Excess unpaired B, gamma, or delta chains
Gamma tetramers in newborns
Beta tetramers in adults
What causes anemia in B-thalassemia?
Not enough B-chains
Excess unpaired a-chains
Anisocytosis
Varying sizes of RBCs
Glucose-6-Phosphate Dehydrogenase Deficiency
Oxidant Exposure causes increased perioxides and premature cell lysis
Bite Cells/Heinz Bodies
G6PD Pathway
G6PD keeps NADP in its reduced state of NADPH so that GSSG can be reduced to GSH in order to get rid of free radicals
G6PD RBCs Lyse because…
Heme breaks away from globin
Heinz Bodies
Denatured globin that sticks to the RBC membrane
Highest incidence of G6PD coincides with what disease?
Malaria
What are the triggers of G6PD deficiency?
Fava Beans, Drugs, Antibiotics, Aspirin, Peroxide
Microangiopathic Hemolytic Anemia
Physical Trauma to RBC
Schistocytes
Triangulocyte
Secondary to other cause
Causes of Microangiopathic Hemolytic Anemia (MAHA)
Runner’s Anemia
Artificial Heart Valve
Anything causing tiny blood clots (fibrin) all over the body in narrow blood vessels (DIC, TTP, HUS)
Normocytic Anemias are caused by…
Blood Loss
Chronic Disease
Kidney or Liver Disease
Aplastic Anemia
Anemia of (acute) Blood Loss
Traumatic Cause
Initially, Hemoglobin is NORMAL
After 2-3 days, see Reticulocytes
Anemia of Chronic Disease
Infections, Inflammation, Malignancy
Iron Metabolism Disturbed
Why is there disturbed iron metabolism seen in Anemia of Chronic Disease?
Absorption is ok, but release of iron is disrupted
Can’t get iron into Hemoglobin and Increased Hepcidin levels leads to short RBC life
Labs for Anemia of Chronic Disease
Low Iron, TIBC
HIGH FERRITIN
High Marrow Storage Iron
Labs for Iron Deficiency Anemia
Low Iron, Ferritin, Marrow Storage Iron
High TIBC
Cause of Anemia of Renal Disease (end stage)
Lack of Erythropoietin
See Echinocytes
Management of Severe Anemia of Renal Disease
Erythropoietin or Blood Transfusion
Anemia of Liver Disease
See acanthocytes and Target Cells
Due to multiple causes
Acanthocytes
Spur Cells - tiny spur-like projections
Echinocytes
Burr Cell, serrated edges over entire surface, sea urchin
Uncomplicated Anemia of Liver Disease (RARE!)
Decreased RBC survival and Impaired Marrow Response
Mild Anemia
Normocytic (sometimes macrocytic)
Poikilocytosis
Complicated Anemia of Liver Disease
Megaloblastosis due to folate deficiency
Microcytosis due to iron deficiency
Aplastic Anemia
Normocystic
Pancytopenia
Marrow not responding
Empty blood/bone marrow
Pancytopenia
Shortage of all blood cell types
Causes of Aplastic Anemia
Idiopathic Drugs Viruses Pregnancy Fanconi Anemia
Clinical picture of Aplastic Anemia
Pallor, Dizziness, Fatigue (Anemia)
Recurrent Infection (Leukopenia)
Bleeding/Bruising (Thrombocytopenia)
Treatment of Aplastic Anemia
From least to most invasive: Avoid further exposure Blood Products G-CSF, Prednisone, ATG Bone Marrow Transplant
