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Board Review - C - Heme/Oncology > Heme/Onco III > Flashcards

Heme/Onco III Flashcards

1
Q

eosinopenia

A

cushing

corticosteroids

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2
Q

corticosteroid

A

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

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3
Q

porphyria

A

defect heme synthesis - accumulation of precursors

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4
Q

lead inhibit what enzymes

A 
ferrochelatase (mito)
ALA dehydratase (cytoplasm)
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5
Q

acute intermittent porphyria

A

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

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6
Q

rate limiting step heme synthesis

A

D-ALA synthase
-cofactor B6

in mitochondria

D-ALA acid then to cytoplasm

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7
Q

porphyria cutanea tarda

A

uroporphyrinogen decarboxylase deficient
-cytoplasm

accumulation - uroporphyrin - tea colored urine

blostering cutaneous photosensitivity

MC porphyria

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8
Q

iron poisoning

A

peroxidation of membrane lipids

chelate - deferoxamine and dererasirox (oral)

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9
Q

PT test

A

extrinsic pathway

1, 2, 5, 7, 10

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10
Q

PTT test

A

intrinsic pathway

all factors - except 7 and 13

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11
Q

hemo A

A

X - linked recessive

factor 8

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12
Q

hemo B

A

X-linked recessive

factor 9

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13
Q

hem C

A

X-linked recessive

factor 11

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14
Q

hemarthroses, bleeding after trauma

A

hemophilia

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15
Q

tx hemophilia

A

desmopressin and factor VIII - A
factor IX - B
factor XI - C

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16
Q

vit K deficient

A

coag defect - normal bleeding time

decreased 2, 7, 9, 10 - protein C and S

17
Q

platelet disorder

A

mucous membrane bleeds, epistaxis, petechiae

long bleeding time

18
Q

bernard soulier

A

Gp Ib

defect platelet plug formation

19
Q

glanzmamm

A

Gp IIb/IIIa

defect plateletaggregation

20
Q

immune thrombocytopenia

A

anti GpIIb/IIIa Abs

splenic macro consumption of Abs

with viral illness

see increased megakaryocytes
tx - steroid - IVIG

21
Q

thrombotic thrombocytopenic purpura

A

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
Q

pentad of TTP

A

neuro and renal sx
fever
thrombocytopenia
microangiopathc hemolytic anemia

23
Q

vWD

A

decreased vWF
-carrier of factor VIII - see PTT longer

and long bleeding time - platelet defect

24
Q

tx vWD

A

desmopressin

25
Q

DIC

A

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
Q

antithrombin deficiency

A

no increase PTT after heparin admin

hypercoag

27
Q

factor V leiden

A

mutant factor 5 resistant to protein C inactivation

hypercoag

28
Q

protein C and S deficiency

A

no inactivation factor 5 and 8

more thrombotic skin necrosis

29
Q

tissue necrosis after warfarin

A

protein C or S deficiency

30
Q

prothrombin gene mutation

A

3’ untranslated region

more prothrombin

higher plasma levels - and venous clots

31
Q

packed RBC

A

Hb and O2 carrying capacity

32
Q

platelets

A

5000 increase per unit

33
Q

fresh frozen plasma

A

clotting factors

warfarin reversal, DIC, cirrhosis

34
Q

cryoprecipitate

A 
fibrinogen
factor 8
factor 13
vWF
fibronectin

tx coag factor deficiency - fibrinogen or factor 8

35
Q

citrate

A

Ca chelator

Board Review - C - Heme/Oncology (4 decks)

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