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49 Cards in this Set
- Front
- Back
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How do you get PNH?
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this is an acquired defect
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Is PNH intrinsic or extrinsic
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Intrinsic- as it is a defect in RBC's cell membrane
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What cell lines are affected in PNH? what is the problem?
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this affect pluripotent stem cells, by a mutation in the GPI anchor gene
When GPI works properly, it inactivates complement- so in PNH- blood cells are sensitive to lysis very easily |
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What are the main clinical signs of PNH? what are they more at risk for later on?
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intravascual hemolysis
hemosidinuria** multiple episodes of venous thrombosis** More at risk for AML |
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What antibody is involved in warm antibody type immunohemolytic anemia?
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IgG
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What are the MAIN causes of Warm antibody type immunohemolytic anemias?
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these are usually secondary effects of: Autoimmune disorders (yes, it IS lupus)
Lymphomas/leukemias Drugs (penicillin) |
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What are the main causes of COLD agglutinin type immunohemolytic anemia?
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Actue- due to infection like mono/mycoplasm
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What is the diagnostic test for immunohemolytic anemias?
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direct coombs test
the temp is used to help specify the class |
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with WAHA, what antibody type is this? and what are the antibodies directed against?
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this is IgG- like all warm hemolytic anemias
and it is directed against Rh blood group antigens |
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What is the clincal sign of WAHA?
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spheroidal transformation of RBC's
Sequestration of RBCs in spleen-> splenomegaly |
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What antibodies is implicated in Cold agglutinin immune hemolytic anemia? and when does this happen?
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caused by IgM
occurs during recovery of some infections like mono/ mycoplasma pneumonia |
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What two things cause hemolytic anemia due to trauma?
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artifical/mechanical valves in the heart
Microangiopathic hemolytic anemia- as this caused mechanical squeezing damage |
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What is the morphology of hemolytic trauma anemia?
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RBC fragments, burr cells, triangle cells
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What is the common defect of all megaloblastic anemias?
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impaired DNA synthesis
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Lack of what two coenzymes leads to megaloblastic anemias?
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B12 and Folate
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What is the morphology of megaloblastic anemias?
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Macrocytic hyperchromic RBC's
Low reticulocyte count Large hypersegmented neutrophils*** |
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What happens to the bone marrow in megaloblastic anemia?
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hypercellular, M:E ratio is now 1:1, instead of usual 3:1.
this means that there are many more erythroid cells (RBCs) trying to be made |
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what type of erythropoiesis is megaloblastic anemias?
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this is Ineffective! trys hard to make RBC's, but they are destroyed prematurely
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What is the defining characteristic of B12 deficiency?
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Anemia with peripheral neuropathy.
(megaloblastic anemia, of course) |
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Does folate deficiency cause neuropathy?
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No it does not
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What is the root cause of pernicious anemia?
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atrophic gastritis with failure to produce intrinsic factor
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What are the clinical signs of Pernicious anemia?
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Shiny, glazed, beefy tongue
Stomach shows diffuse chronic gastritis megaloblastic anemia |
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What causes subacute combined degeneration?
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Pernicious anemia due to B12***
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What causes spastic paraparesis, sensory ataxia, and severe parasthesia in lower limbs?
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lack of B12-> pernicious anemia
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What are the 3 clinical diagnostic factors for pernicious anemia (what she'll put on test)
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Megaloblastic anemia
hypersegmented neurtophils neurologic changes |
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What is someone more at risk for with pernicious anemia?
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these people are at risk for gastric cancer
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What are the three major causes of folate deficiency?
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decreased intake
increased requirements- like pregnancy, bleeding, cancer impaired used of folate- some drugs like phenytoin can impair it, methotrexate (cancer drug) impairs DHF reductase |
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How is folate deficiency anemia diagnosed?
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megaloblastic anemia with OUT neruopathy
decreased folate levels in serum or RBC's |
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When thinking of folate deficiency, what other diagnosis must be excluded?
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vitamin B12 deficiency, which can lead to neruopathies
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What is the iron storage molecule?
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ferritin
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What is the iron transport molecule in the blood?
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transferrin. easy
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what is a good indicator of the adequacy or iron stores?
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ferritin
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What is the typical total iron binding capacity? why molecule is it?
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this is transferrin, typical total iron binding capacity is 300-350 micrograms
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What does hepcidin do?
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this blocks the absorption of iron into storage cells
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What kind of iron is most easily absorbed?
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heme iron....so vampires will never be anemic
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what are the four main causes of iron deficiency anemia?
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lack of iron in diet
impaired absorption increased need chronic blood loss |
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iron deficiency in adult men and postmenopausal women should always be considered...
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as a GI bleed until proven otherwise
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What type of anemia does iron deficiency produce in a blood smear?
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microcytic, hypochromic red cells
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What is Plummer-Vinson syndrome?
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microcytic hypochromic anemia with
atrophic glossitis and esophageal webs |
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What are the labs like for iron deficient anemia?
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low hgb, hct
low serum iron, low ferritin total iron binding capacity is high low transferrin saturation |
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What are the labs like for anemia of chronic disease?
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Low serum iron
reduced total iron binding capacity abundant stored iron in MP cells. shows as a defect in reuse of iron (maybe hepcidin related?) |
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What is aplastic anemia?
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this is a pancytopenia with anemia, neutropenia, thrombocytopenia
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What causes aplastic anemia?
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failure or suppression of stem cell lines
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What are the two main causes of aplastic anemia?
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exposure to drugs or chemicals
or radiation |
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How can aplastic anemia occur after viral infections?
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immunologically mediated supression
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What is the morphology of aplastic anemia?
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the bone marrow is hypocellular, mainly has fat cells in it- but no hematopeoitc cells
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What usually causes pure red cell aplasia?
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usually caused by a neoplasm- like thymoma, leukemia, or can be autoimmune
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What is myelophthisic anemia?
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this is a space occupying lesion in the bone marrow- that takes up space usually used for erythropoesis- affect all cell lines
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what usually causes myelophthisic anemia?
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metastatic cancer of breast, lung, prostate, thyroid and adrenal glands
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