Heme Flashcards
increased band cells
increased myeloid proliferation
CML, bacterial infections
CD14
surface marker for macrophages
eosinophils produce
histaminase
arylsulfatase
eosinophil defend against
neoplastic asthma allergic collagen vascular dz parasites
basophil
allergic rxn
heparin, histamine, leukotrienes
Dendritic cells
express MHC class II and Fc receptor called Langerhan's cells in skin
lymphocytes
divide into B and T cells
Leukocytes
granulocytes –> basophil, neutrophil, eosinophil
mononuclear - monocytes, lymphocytes
Kallikrein
activates bradykinin
ACE
inactivates bradykinin
Warfarin
inhibits epoxide reductase
Vit K def
decreased synthesis of 2,7,9,10, protein C and S
Heparin
activates antithrombin
antithrombin
inhibits 2,7,9,10,11,12
protein c
inactivates V
V
prothrombin to thrombin
thrombin
fibrinogen to fibrin monomers
Ticlopidine
inhibits ADP induced expression of GpIIb/IIIa
Clopidogrel
inhibits ADP induced expression of GpIIb/IIIa
decreased ESR
polycythemia
sickle cell
CHF
hypofibrinogenemia
4 gene deletion of alpha
excess gamma-globin –> forms Hb barts
hydrops fetalis
HbH disease
3 gene deletion of alpha
excess beta, B4 –> HbH
cis alpha deletion
asian
trans alpha deletion
african
B thal minor
asymptomatic
increase HbA2
B thal major
severe anemia requiring blood transfusion marrow expansion --> crew cut --> skeletal deformitins increased HbF (alpha2gamma2)
HbS/B thal
mild to moderate sickle cell dz depending on amount of B globin produced
lead inhibits
ferrochelatase and ALA dehydratase
decreases heme synthesis
treatment for lead poisoning
dimercaprol
EDTA
sideroblastic anemia
x linked defect in ALA synthase gene
treatment for sideroblastic anemia
pyroidoxine
ACD
increased hepcidin
increased ferritin
Causes of aplastic anemia
rdiation, drugs
viruses
Fanconi
Hereditary Spherocytosis Cause
defect in RBC membrane, ankryin, spectrin
Paroxysmal noctural hemoglobinuria triad
hemolytic anemia
pancytopenia
venous thrombosis
PNH Cause
impaired synthesis of GPI anchor/CD55,59
HbS mutation
glutamic acid —> valine
Warm Autoimmune hemolytic anemia
IgG
SLE, CLL, alpha-methyldopa
Cold Autoimmune hemolytic anemia
IgM
Mycoplasma, Mono, CLL
Microangiopathic anemia
DIC, TTP-HUS, SLE, malignant HTN
macroangiopathic anemia
prosthetic heart valves
aortic stenosis
Acute Intermittent Porphyria
uroporphyrin in urine
painful abdomin, port wine colored urine, polyneuropathy, psych disturbances, precipitated by drgs
Acute Intermittent Porphyria treatment
glucose and heme (inhibit ALA synthase)
PT test
extrinsic pathway
1,2,5,7,10
PTT
intrinsic
all except 7 and 13
Bernard-Soulier syndrome
defect in platelet plug formation, decreased GpIb
decreased platelet count
increased bleeding time
Glanzmann’s thrombasthenia
decreased GpIIb/IIIa
increased bleeding time only