Flashcards in Red Blood Cell disorders Deck (26):
underlying cardiac/circulatory problems; responds to diuretics
due to antibodies against white blood cells, leading to a breakdown of the walls of blood vessels in the lungs, allowing fluid to infiltrate the lungs; it does not respond to diuretics
Anemia of chronic disease
spherocytes are seen in?
-warm autoimmune hemolytic anemia?
blister/basket cells are seen in?
-glutathione synthetase deficiency
Howell-Jolly bodies are composed of ___ and seen in diseases like___. They are indicative of ____
hereditary spherocytosis s/p splenectomy
Heinz bodies are made of ___ and seen in ___
Schistocytes are ___ and seen in ___
-microangiopathic hemolytic anemias (HUS, TTP, possibly DIC)
-brute force trauma - malignant HTN, mechanical heart valves and pumps
TTP is due to:
deficiency of VWF cleaving enzyme (ADAMTS13)
Steps of B12 absorption
-B12+food, B12 released
-B12+salivary proteins (R binders/cobalophilins), released by proteases
-B12+Intrinsic Factor --> ileum
-B12+transcobalamin II --> plasma --> proliferating cells, marrow
sideroblastic anemia occurs due to defective ____ (enzyme)
Acute Intermittent Porphyria occurs due to defective ____ (enzyme); results in accumulation of _____
porphobilongen deaminase; porphobilinogen
Porphyria cutanea tarda occurs due to defective ____ (enzyme); results in accumulation of ____.
It is managed by what three things? And how do these each help?
uroporphyrinogen decarboxylase; uroporphyrinogen
phlebotomy - removes iron, which oxidizes uroporphyrinogen to uroporphyrin and other things that can inhibit uroporphyrinogen decarboxylase
sunscreen - skin
chloroquine - complexes uroporphyrin to be excreted in urine
____ catalyzes the rate-limiting step of heme synthesis. It also requires ___ cofactor
ALA synthase; vitamin B6 (pyridoxal phosphate is active form)
ALA synthase is stimulated by ____ and inhibited by ____
ETOH, barbiturates; heme
What two enzymes in the heme synthesis pathway are inhibited by heavy metals?
ALA synthase; ferrochelatase
inhibits Vitamin K epoxide reductase, prevent Vit K recycling and thus inhibiting functional serine proteases and proteins C/S
Has initial procoagulant effect since it the anticoagulant proteins C/S are affected first due to their short half lives --> hypercoagulability and skin necrosis
binds antithrombin to make it more active in
indicated for short term prophylaxis and treatment of venous thrombosis, PE, and some coagulopathies
Binds platelet ADP receptors
How would Vitamin K deficiency affect PT and PTT?
What factors does Vit K affect?
PT increased because factor VII has a short half life
PTT would be normal except for when severe Vit K deficiency
Vit K gamma carboxylates all the serine proteases (2, 7, 9, 10, 11) and proteins C/S
is like TTP but in the kidneys
triggered by shiga-like toxin, E.coli O157:
immune mediated thrombocytopenia
due to IgG autoantibodies against platelet surface glycoproteins
acute - in children often after viral infection, self-limiting
chronic - in adults
Direct fibrinolytics target ___?
HIT (heparin induced thrombocytopenia) occurs ____ days after heparin exposure.
Describe the etiology?
5-14 days (faster timing if exposed before in the last 90 days)
Platelet Fc receptor binds heparin-PF4 complex, causing indiscriminate platelet activation wherever these complexes are
Describe the etiology and diagnosis of Antiphospholipid Syndrome?
Antiodies against epitopes of plasma proteins bound to phospholipids (lupus anticoagulant, anticardiolipin antibodies, antibeta2 GP1 antibodies)
Must have positive antiphospholipid test once and again after at least 12 weeks
some clinical manifestation of thrombosis