Flashcards in Heme/Onco III Deck (35)
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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
