Heme/Onco III Flashcards
eosinopenia
cushing
corticosteroids
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
porphyria
defect heme synthesis - accumulation of precursors
lead inhibit what enzymes
ferrochelatase (mito) ALA dehydratase (cytoplasm)
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
rate limiting step heme synthesis
D-ALA synthase
-cofactor B6
in mitochondria
D-ALA acid then to cytoplasm
porphyria cutanea tarda
uroporphyrinogen decarboxylase deficient
-cytoplasm
accumulation - uroporphyrin - tea colored urine
blostering cutaneous photosensitivity
MC porphyria
iron poisoning
peroxidation of membrane lipids
chelate - deferoxamine and dererasirox (oral)
PT test
extrinsic pathway
1, 2, 5, 7, 10
PTT test
intrinsic pathway
all factors - except 7 and 13
hemo A
X - linked recessive
factor 8
hemo B
X-linked recessive
factor 9
hem C
X-linked recessive
factor 11
hemarthroses, bleeding after trauma
hemophilia
tx hemophilia
desmopressin and factor VIII - A
factor IX - B
factor XI - C
vit K deficient
coag defect - normal bleeding time
decreased 2, 7, 9, 10 - protein C and S
platelet disorder
mucous membrane bleeds, epistaxis, petechiae
long bleeding time
bernard soulier
Gp Ib
defect platelet plug formation
glanzmamm
Gp IIb/IIIa
defect plateletaggregation
immune thrombocytopenia
anti GpIIb/IIIa Abs
splenic macro consumption of Abs
with viral illness
see increased megakaryocytes
tx - steroid - IVIG
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
pentad of TTP
neuro and renal sx
fever
thrombocytopenia
microangiopathc hemolytic anemia
vWD
decreased vWF
-carrier of factor VIII - see PTT longer
and long bleeding time - platelet defect
tx vWD
desmopressin
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
antithrombin deficiency
no increase PTT after heparin admin
hypercoag
factor V leiden
mutant factor 5 resistant to protein C inactivation
hypercoag
protein C and S deficiency
no inactivation factor 5 and 8
more thrombotic skin necrosis
tissue necrosis after warfarin
protein C or S deficiency
prothrombin gene mutation
3’ untranslated region
more prothrombin
higher plasma levels - and venous clots
packed RBC
Hb and O2 carrying capacity
platelets
5000 increase per unit
fresh frozen plasma
clotting factors
warfarin reversal, DIC, cirrhosis
cryoprecipitate
fibrinogen factor 8 factor 13 vWF fibronectin
tx coag factor deficiency - fibrinogen or factor 8
citrate
Ca chelator
