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37 Cards in this Set
- Front
- Back
Hereditary spherocytosis
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autosomal dominant trait
abnormal RBC membrane due to a protein and lipid defect. Reduced surface area, impaired flexibility and increased Na permeability Increased MCHC Increased osmotic fragility |
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Hereditary elliptocytosis
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autosomal dominant trait
defect inteh membrane cytoskeleton greater than 25% ellipotocytes extravascular hemolysis |
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jaundice
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increased extravascular hemolysis
indirect conjugated bilirubin |
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extravascular hemolysis
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shape problem not hgb problem
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pyruvate kinase deficiency
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rare
autosomal recessive enzyme deficiency of the EM pathway burr cells reduces ATP production needed for NA/K pump |
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glucose 6 phosphate dehydrogenase deficiency
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inherited
doesnt appear without a oxidant stressor x linked recessive red cell enzyme deficiency causing hemolysis intravascular hemolysis heinz bodies/bite cells |
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Hemoglobin S (sickle cell anemia)
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inherited
substitution of valine for glutamic acid in the beta chains of the Hgb molecule homozygous (SS) sickle cell disease heterozygous (AS) sickle cell trait intra- and extravascular hemolysis Targets |
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HGB SC
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inherited
2 hgb varients targets pocketbook cells mild anemia if at all |
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HGB C
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targets
hgb c crystals mchc increased usually no symptoms can become micro/hypo because body keeps taking cells out of circulation and producing more then run out of iron |
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paroxsmal nocturnal hemoglobinuria
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rare
acquired intrinsic blood pH drops at night and binds complement cells lyae in the morning extra- and intravascular hemolysis biochemical and membrane abnormality low hgb and platelet hams acified serum test or the 'sugar H2O' test |
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heinz bodies
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denaturation and precipitation of unstable hgb
pitted golf ball supravital staining |
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microangiopathic hemolytic anemias
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a disease of small blood vessels (changes within the small vessels cause circulating RBCs to be hemolyzed/fragmented)
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DIC
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occurs in patches
inside vessels clotting fibrin clots form inside vessels and RBCs are fragmented as they push through the the clots |
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TTP
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rare
extensive platelet clumping occurs within blood vessels leading to decresed plts and schisto causes renal failure |
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HUS
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infants and kids
intravascular hemolysis renal failure |
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p. falciparum
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rare
may be more than one ring causes hemolysis the fastest maurere dots may be present crescent gamecytes (bannana shaped) |
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p. vivax
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rare
four maturation stages rings trophozoited schizonts gametocytes schuffners granules or stippling |
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isoantibodies/alloantibodies
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specific antigen from an individual of the same species
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autoantibodies
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reacts with self antigens
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DAT
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differentiates immune hemolytic anemias from non immune hemolytic anemias
detects the presence of antibodies that are attached to the Ags on RBC membrane |
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immune hemolytic anemia
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Abs that attach to Ags on RBC membranes
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fanconis
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inherited aplastic anemia
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idiopathic aplastic anemia
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cause unknown 70% of cases
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secondary aplastic anemia
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acquired 25% of cases
medication, radiation |
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pure red cell aplasia
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rare
N/N inherited or acquired normal leukocytes and platlet counts adn a marked decrease in marrow erythroblasts |
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megaloblastic macrocytic anemia
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abnormal development of cell nuclei (DNA synthesis)a as cells mature in the bone marrow
marco/oval cytoplasm more immature nuclei large chromatin never becomes clumped nucleoli are retained hypersegmented neutrophils |
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vitamin B12 deficiency
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meat and dairy
3-6 years worth in storage requires for a single critical reaction during normal DNA synthesis become deficient by defective production of intrinsic factor, nutritional deficiency, competition for dietary B12(bacteria), malabsorption hypersegmented neutrophils |
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folate deficiency
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leafy greens, liver, kidney, fruit, dairy
3 months worth in storage required for three reactions that lead do DNA synthesis(involving amino acid synthesis- purines and pyrimidines) folate needs B12 to work become deficient by short supply, impaired absorption, increased need, impaired utilization(alcohol), excessive loss hypersegmented neutrophils |
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pernicious anemia
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lack of intrinsic factor
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ineffective erythropoiesis
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increased erythrocyte precursors in the bone marrow but decreased release into the peripheral blood.
decreased reticulocytes |
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ineffective granulopoiesis
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abnormal formation of leukocytes
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ineffective thrombosis
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abnormal formation of plt with decreased plt life span
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iron deficient anemia
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body iron stores are inadequate to preserve homeostasis
caused by increased demand, inadequate intake, chronic blood loss |
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anemia of the chronic disease
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destructing faster than the bone marrow can produce
caused by a chronic disease, infection, inflammation, malignancy iron is trapped in the macrophages unable to compensate |
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sideroblastic anemia
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accumulation of excess iron from increased iron storage in macrophages in various tissue
hereditary =rare acquired - most common lead poisoning lead injures the RBC membrane and blocks at least three enzymes in the heme synthesis pathway (slows down the breakdown of RNA= basophilic stippling) |
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thal
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inherited
defect in the production of alpha and beta globin chains |
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alpha thal
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defect in the synthesis of alpha chain
silent carrier 1 gene deletion alpha thal minor- 2 gene deletion HbH disease- 3 gene deletion Barts hydrops fetalis 4 gene deletion |