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20 Cards in this Set
- Front
- Back
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Blood Cell Normal Life Span
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120 days
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Hereditary Spherocytosis
pathogenesis? genetic inheritance pattern? |
defect in RBC membrane - usually due to
ankyrin - binds spectrin to membrane spectrin - cytoskeletal molecule round cells well get sheared in spleen -> hemolysis autosomal dominant mostly |
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Hereditary Spherocytosis
complications |
viral illness or medication can set off aplastic crisis
megaloblastic crisis - if lack folate (during pregnancies) bilirubin gallstones ulcers, dermatitis due to undeformable RBC |
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Hereditary Spherocytosis
diagnosis? treatment? |
diagnosis: RBC osmotic fragility test
treatment: splenectomy if severe symptoms, take folic acid |
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Hereditary Elliptocytosis
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defect in alpha and beta spectrin or some others
multiple types |
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Hereditary pyropoikilocytosis
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Deformed RBCs
severe hemolytic anemia due to increased temperature sensitivity of hemoglobin - do heat study MCB low usually african american |
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Acanthocytosis
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seen with advanced liver disease
elevated cholesterol in outer leaflet expands the outer membrane surface -> spiky appearance |
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Abetalipoproteinemia
pathogenesis? genetic inheritance pattern? symptoms |
increased membrane sphingomyelin as the RBC ages causes expansions of outer leaflet of bilayer - acanthocytosis
autosomal recessive symptoms: retinitis pigmentosa, neurologic abnormalities |
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Stomatocytosis
morphology what diseases cause |
RBCs look like a mouth
inherited: due to cation permeability changes -hydrocytosis -xerocytosis acquired: rh deficiency, acute alcoholism, malignant neoplasms, CV and hepatobiliary disease |
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Glucose 6-phosphate dehydrogenase (G6PD) deficiency
-A- form |
disorder of the hexose monophosphate shunt
G6PD needed to protect RBC from oxidative damage triggered by fava beans, drugs, etc. no hemolysis until exposed to a trigger cannot hemolize to death because have some population of normal enzyme presents with hematouria protects against malaria one of two types of G6PD deficiency |
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G6PD - mediterranean
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G6PD in mediterranean, ashkenazi jews, asians
very unstable form of protein - functional one time, other time not chronic hemolysis, splenomegaly, icterus |
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G6PD deficiency clinical aspects
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Decreased G6PD levels
heinze bodies bite cells spherocytes increased reticulocytes decreased G6PD level |
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Heinze bodies
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stains in a crystal violet stain - denatured hemoglobin in RBC
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Warm antibody autoimmune hemolytic anemia (AIHA)
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autoantibodies IgG react with membrane at 37degrees C
usually some underlying disease - CLL, lymphoma, SLE, ovarian cancer back pain, malaise, dark urine, jaundice, splenomegaly, hepatomegaly, LN enlarged |
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warm antibody autoimmune hemolytic anemia (AIHA) diagnosis
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direct coombs test
IgG antibodies targeting Rh blood group antigens |
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cold agglutinin syndrome
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cold temperature activated autoantibodies->
hemolysis and vascular sludging acrocyanosis, hemoglobinuria direct coombs test positive for C3d, IgM autoantibody detectable in serum see with underlying disease: mycoplasma penumoniae, mono, CLL, HD, lymphoma |
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cold agglutinin syndrome diagnosis
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direct coombs test positive for C3d, IgM autoantibody detectable in serum
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paroxysmal cold hemoglobinuria
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IgG attaches during cold, fixes complement when warmed - no hemolysis until warmed
associated with syphilis, viral infection Direct Coombs negative |
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drug induced AIHA
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long exposure to drug causes immune system to create antibodies to the drug which cross react with RBCs
drug doesnt have to be present to make autoantibodies direct coombs positive |
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paroxysmal nocturnal hemoglobinuria
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chronic hemolysis caused by a clonal stem cell disorder that affects all cell lines
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