Term
| Def of anemia in men & women |
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Definition
Men <13.5
Women <12.5
Polycythemia: M>17.5; W>16.0 |
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Term
| name the 4 broad categories of microcytic anemias |
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Definition
remember, microcytic anemias are problems w/ hemoglobin production: problem w/ either decreased Fe, ineffective protoporphyrin synthesis, or globin synthesis
4 major types:
1) Fe deficiency
2) Anemia of chronic disease (de facto Fe deficiency)
3) Sideroblastic anemia (problem w/ protoporphyrin synthesis)
4) Thalassemias (problem w/ globin synthesis) |
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Term
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Definition
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Term
Receptor on enterocytes that is downregulated by Hepcidin?
On the luminal membrane, there is a Heme transporter & a non-heme transporter. What is the non-heme transporter called? |
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Definition
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Term
| In both Fe-deficiency & anemia of chronic disease. What type of anemia exists before the microcytic-hypochromic anemia? |
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Definition
| normocytic, normochromic (bone marrow makes normal size cells, just fewer) |
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Term
Ferritin, TIBC, %Saturation, Serum Fe, & free erythrocyte protoporphyrin:
- Fe-deficiency anemia
- Anemia of chronic disease |
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Definition
-Fe def: ferritin, %Sat, Serum Fe = decreased; TIBC = increased
- AOCD: ferritin = increased; %Sat, Serum Fe, TIBC = decreased
Free erythrocyte protoporphyrin is increased in both |
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Term
| Ferrochelatase step in heme synthesis is carried out in what part of the cell? |
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Definition
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Term
| name 3 causes of sideroblastic anemia: |
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Definition
- Alcoholism (mitochondrial poison)
- Lead poisoning (inhibit ALA dehydrogenase & Ferrochelatase)
- B6 deficiency (required for ALA synthase; think INH Tx) |
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Term
| Ferritin, TIBC, %sat, & serum Fe in sideroblastic anemia |
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Definition
Ferritin: increased
TIBC: DECREASED
%sat: increased
Serum Fe: Increased
These values are identical to that of an iron overloaded state (e.g. hemochromatosis) |
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Term
| How many alpha chains must be deleted to see HbH? Hemoglobin barts? |
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Definition
HbH: three alpha's deleted (person can live but is severely anemic)
Barts: all 4 deleted; hydrops fetalis |
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Term
| increased HbF & HbA2 in alpha or beta thal? |
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Definition
| beta thal (remember you need the alpha chain for all the different types of hemoglobin. HbH is 4 beta chains seen in severe alpha thal) |
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Term
| Alpha or beta thal: severe extramedullary hematopoiesis & expansion of medullary spaces creating 'crew-cut' & 'chipmunk facies' |
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Definition
Beta thal; beta thal is also at sever risk for aplastic crisis w/ B19 infection
beta thal has ineffective erythropoiesis (probably because alpha4 tetramer is less soluble than HbH)
This can also be seen with sickle-cell anemia |
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Term
Name etiologies of macrocytic anemia:
- With hypersegmentation (i.e. megaloblastic change)
- Without megaloblastic change |
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Definition
- folate or B12 deficiency
- Alcoholism, liver disease, drugs (e.g. 5FU) |
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Term
| what cell has intrinsic factor |
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Definition
| parietal cells (remember the "P's" of parietal cells: Pink, Proton pump, Pernicious anemia) |
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Term
| Why does pancreatic insufficiency cause B12 deficiency? |
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Definition
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Term
| What effect of megaloblastic anemia caused by folate or B12 deficiency causes increased risk for thrombosis? |
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Definition
increased homocysteine
homocysteine may alos increase risk for developing atherosclerosis |
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Term
| % of reticulocytes that qualify as an appropriate response to anemia? |
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Definition
>3%
reticulocyte count must be corrected for the amount of anemia: multiply by Hct/45 |
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Term
| Howell-Jolly bodies & Heinz bodies. Which one do you need a supravital stain to see? |
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Definition
Need a supravital stain to see Heinz bodies (G6PD, clumps of precipitated Hb); Spleen will remove these and cause "bite cells"
Howell jolly seen with asplenia (fragments of nuclear material)
ASIDE: basophilic stippling is clumped rRNA seen in "TAIL": Thalassemias, ACD, Iron def anemia, Lead poisoning |
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Term
| 2 conditions you see increased MCHC in? |
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Definition
| hereditary spherocytosis & warm immune hemolytic anemia |
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Term
For sickle cell disease
1) classical presenting sign (~6mo old)
2) most common cause of death in kids
3) most common cause of death in adults |
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Definition
1) dactylitis
2) encapsulated organisms (S.pneumo/H.flu sepsis; Salmonella osteomyelitis)
3) Acute chest syndrome |
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Term
| Abnormal Hb migration of electrophoresis & crystals seen on peripheral smear? |
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Definition
HbC disease
Lysine for arginine substitution (Hemoblobin SEE has lySEEn |
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Term
paroxysmal nocturnal hemoglobinuria:
- Etiology (hereditary? acquired?)
- mechanism of cell lysis
- Cancer association |
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Definition
- acquired defect in glycosylphosphatidylinositol (GPI, CD55) --> Decay accelerating factor not attached to cell surface --> destruction of cell by complement
- Complement lysis (esp @ night w/ transient acidosis)
- AML (10% of pts will develop this; robbins says something about selection due to immune surveillance etc)
can also get Fe-deficiency anemia from losing Fe in urine all the time |
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Term
| main cause of death in paroxysmal nocturnal hemoglobinuria? |
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Definition
thrombosis (destroyed platelets-same mech as RBC destruction-release cytoplasmic contents into circulation, inducing thrombosis)
NOTE: PND causes hemolysis of RBCs, platelets, & WBCs |
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Term
| intravascular hemolysis that presents as back pain & hemoglobinuria |
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Definition
G6PD deficiency
back pain b.c. Hb is nephrotoxic |
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Term
For immune hemolytic anemias: IgG & IgM
1) Warm type? cold type?
2) SLE, CLL, Penicillin/cephalosporin, & methyldopa associate?
3) Mycoplasma pneumonia associate?
4) intravascular/extravascular hemolysis? |
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Definition
1) IgG = warm; IgM = cold (i.e. attaches & fixes complement in extremities
2) IgG warm type; Pen/Cephs attach to RBC & create an antigenic protein-drug complex; Antibodies directed against methyldopa have a X-reaction w/ RBCs
3) IgM (cold)
4) IgG = extravascular; IgM = intravascular
NOTE: cold type associated w/ raynaud's phenomenon; Robbin's says that the cold type is extravascular hemolysis, but there is "paroxysmal cold hemoglobinuria" that has IgG that binds & lyses cells in extremities in cold weather) |
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Term
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Definition
1) Hemolytic anemia (microangiopathic --> helment cells)
2) Elevated liver enzymes
3) Low platelet count |
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