Flashcards in Heme/Onco III Deck (35)
1
eosinopenia
cushing
corticosteroids
2
corticosteroid
decreased activation of neutrophil adhesion molecule
impair migration out of vasc
sequester eosinophil in lympho node - cause to die
high neutro
low eosino and lympho
3
porphyria
defect heme synthesis - accumulation of precursors
4
lead inhibit what enzymes
ferrochelatase (mito)
ALA dehydratase (cytoplasm)
5
acute intermittent porphyria
porphobilinogen deaminase deficient
-cytoplasm enzyme
accumulation - porphobilinogen, d-ALA, coporphobilinogen
pain abdomen
port wine urine
polyneuropathy
psych disturbance
precipitated by drugs, alcohol, staration
tx - glucose and heme - inhibit ALA synthase
6
rate limiting step heme synthesis
D-ALA synthase
-cofactor B6
in mitochondria
D-ALA acid then to cytoplasm
7
porphyria cutanea tarda
uroporphyrinogen decarboxylase deficient
-cytoplasm
accumulation - uroporphyrin - tea colored urine
blostering cutaneous photosensitivity
MC porphyria
8
iron poisoning
peroxidation of membrane lipids
chelate - deferoxamine and dererasirox (oral)
9
PT test
extrinsic pathway
1, 2, 5, 7, 10
10
PTT test
intrinsic pathway
all factors - except 7 and 13
11
hemo A
X - linked recessive
factor 8
12
hemo B
X-linked recessive
factor 9
13
hem C
X-linked recessive
factor 11
14
hemarthroses, bleeding after trauma
hemophilia
15
tx hemophilia
desmopressin and factor VIII - A
factor IX - B
factor XI - C
16
vit K deficient
coag defect - normal bleeding time
decreased 2, 7, 9, 10 - protein C and S
17
platelet disorder
mucous membrane bleeds, epistaxis, petechiae
long bleeding time
18
bernard soulier
Gp Ib
defect platelet plug formation
19
glanzmamm
Gp IIb/IIIa
defect plateletaggregation
20
immune thrombocytopenia
anti GpIIb/IIIa Abs
splenic macro consumption of Abs
with viral illness
see increased megakaryocytes
tx - steroid - IVIG
21
thrombotic thrombocytopenic purpura
inhibition of ADAM TS13 - vWF metalloprotease
decreased degradation vWF multimers
-get platelet adhesion and aggregation
-leads to thrombosis
hemolysis - schistocytes and elevated LDH
tx - plasmapharesis - steroids
22
pentad of TTP
neuro and renal sx
fever
thrombocytopenia
microangiopathc hemolytic anemia
23
vWD
decreased vWF
-carrier of factor VIII - see PTT longer
and long bleeding time - platelet defect
24
tx vWD
desmopressin
25
DIC
widespread activation of clotting
-get deficient clot factors - bleed out
due to sepsis, trauma, obstetric complication, acute pancreatitis, malignancy, nephrotic syndrome, transfusion
see D dimer, schistocytes, fibrinogen, factor V and VIII low
26
antithrombin deficiency
no increase PTT after heparin admin
hypercoag
27
factor V leiden
mutant factor 5 resistant to protein C inactivation
hypercoag
28
protein C and S deficiency
no inactivation factor 5 and 8
more thrombotic skin necrosis
29
tissue necrosis after warfarin
protein C or S deficiency
30
prothrombin gene mutation
3' untranslated region
more prothrombin
higher plasma levels - and venous clots
31
packed RBC
Hb and O2 carrying capacity
32
platelets
5000 increase per unit
33
fresh frozen plasma
clotting factors
warfarin reversal, DIC, cirrhosis
34
cryoprecipitate
fibrinogen
factor 8
factor 13
vWF
fibronectin
tx coag factor deficiency - fibrinogen or factor 8
35