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53 Cards in this Set
- Front
- Back
clinical signs of accumulation of hg degradation products
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jaundice
gallstones dark red urine |
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acute exacerbation of hemolysis due to activation of MACs
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hemolytic crisis
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impairment of BM red cell production
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aplastic anemia
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depletion of nutrients, folic acid, or iron
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marrow exhaustion
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causes hemoglobinemia and hemoglobinurea
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intravascular RBC destruction
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causes of intravascular RBC destruction
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toxic substances in the red cell or environment (infection, drugs)
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mechanism of intravascular RBC destruction
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complement activation
physical trauma (march hemoglobinemia, artificial heart valves, thermal burns) |
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mechanism of extravascular hemolysis
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Ig bind to RBC and they get trapped in the red pulp of spleen by MACs Fc binding
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corrected reticulocyte count in rapid red ell turnover
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>4% (hallmark of acc red cell turnover)
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what is a spherocyte and what disease is it indicative of
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small, hyperchromic red cell; hereditary spherocytosis, autoIg hemolytic anemias, or burns
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diseases that cause schistocyte formation
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HUS
TTP DIC prosthetic heart valves |
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three types of autoimmune hemolytic anemias
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warm
cold paroxysmal cold |
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most common type of autoimmune hemolytic anemia
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warm
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Ig subtype that mediates warm hemolytic anemia
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IgG
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antibody in warm hemolytic anemia is directed at what
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Rh factor
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Ig subtype that mediates cold hemolytic anemia
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IgM
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type of autoimmune hemolytic anemia that usually follows an infection
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cold
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mechanism of RBC destruction in warm antibody
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extra and intravascular
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mechanism of RBC destruction in cold antibody
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extravascular
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Ab against I antigen on RBC
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cold agglutinins
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biphasic autoimmune hemolytic anemia where IgG fixes in the cold and activates complement upon warming
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paroxysmal cold agglutinins
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alloimmune hemolytic anemia of fetus due to Rh incompatibility with mother
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erythroblastosis fetalis (hydrops)
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prophylaxis of hydrops fetalis
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Rh Ig
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what is the direct coombs test
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marker Igs are added to patient serum to look for Igs to RBC
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what is the indirect coombs test
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patient serum is added to lab red cells and look for reaction
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enzyme deficiency that causes hemolyic anemia
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glucose 6 phosphate dehydrogenase deficiency
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heinz bodies
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G6PD
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clinical signs of G6PD
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asymptomatic until drug or illness
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tx of G6PD deficiency
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remove agent and it resolves
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most common enzyme deficiency in glycolysis
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pyruvate kinase
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pyruvate kinase deficiency results in what changes to the RBC
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membrane deformity and extravascular destruction
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etiology of hereditary spherocytosis
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deficiency in cytoskeleton protein spectrin
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only cell that has elevated MCHC
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spherocytes
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therapy to hereditary spherocytosis
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splenectomy
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two types of hereditary hemolytic anemias
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hereditary spherocytosis
hereditary elliptocytosis |
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hemolytic anemia that is exacerbated by sleep
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paroxysmal nocturnal hemoglobinuria
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RBC membrane disorder caused by deficient complement regulation and abnormal sensitivity to complement lysis
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paroxysmal nocturnal hemoglobinuria
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tests for paroxysmal nocturnal hemoglobinuria
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flow cytometry for CD55/59
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acanthocyte
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hemolytic anemia due to hepatocellular disease
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what is march hemoglobinemia
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hemolysis due to RBC trauma
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three most common thrombotic microangiopathies
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DIC
TTP HUS |
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tetrad of sx seen in HUS
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seizures
hemolytic anemia acute renal failure thrombocytopenia |
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etiology of diarrhea positive HUS
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shigella toxin e.coli
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pentad of sx in TTP
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fever
ARF thrombocytopenia microangiopathic HA neurologic sx |
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etiology of TTP
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absence of ADAMTS which leaves large vWF multimers
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fever
chills headache low back pain |
acute hemolysis
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less severe form of mother fetal hemolytic anemia that causes mild anemia
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ABO compatibility where mother is O and child is A or B
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sex linked enzyme deficiency hemolytic anemia
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G6PD
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anemia
venous thrombosis increased infection increased leukemia |
PNH
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membrane disorder caused by an acquired mutation in PIG gene
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PNH
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tx of TTP
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plasma exchange
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peripheral smear findings in hemolytic anemias
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spherocytes
schistocytes |
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reticulocyte levels in all hemolytic anemias
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elevated (reticulocytosis is way to differentiate hemolytic anemias)
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