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(AA) Hemostasis > Section 9 Factor deficiencies > Flashcards

Section 9 Factor deficiencies Flashcards

1
Q

Hemophilia A

A

factor VIII (C)

most common def in US
X linked recessive in males

females asympt
30% new cases have no family hxt

Clinical bleeding

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2
Q

Hemophilia A
ref range
lab results

A

ref range 50-150%

mild 5-30
moderate 1-5
severe <1

PFA norm
PT norm
APTT prolonged

confirm w factor assay

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3
Q

Aquired VIII deficiency

A

DIC: using VIII
Liver disease: unable to produce

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4
Q

Hemophilia B

A

IX deficiency
royal disease
2nd most common

x linked recessive in males

females some bleeding w levels of 35%

bleeding less severe than A

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5
Q

Hemophilia B labs

A

PFA norm
PT norm
APTT prolonged

factor assay to confirm

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6
Q

Aquired hemophilia B

A

DIC/Liver disease

Vit K deficiency
Oral Anticoags

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7
Q

Afibrinogenemia

A

absence or low
hereditary low recessive
Bleeding

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8
Q

Hypofibrinogenemia

A

<100mg
hereditary recessive
Bleeding

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9
Q

Dysfibrinogenemia

A

functionally abnormal
both bleeding and thrombosis

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10
Q

Hyperfibrinogenemia

A

APR

stress increases fibrinogen can lead to thrombosis

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11
Q

Prothrombin Deficiency
common?
what kind of deficiency?
males vs females?

homozygous
heterozygous?

A

II

RAREST congenital deficiency
recessive
equal in males/females

homozygous bleeding
hetero - asympt

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12
Q

Aquired II deficiency

A

DIC/LIVER

Vit K def, oral anticoags

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13
Q

V deficiency
frequency?
homozygous causes?
no…

aquired:

A

1/million
homozygous bleeding
no cryo - not adequet V

aquired: Dic/Liver

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14
Q

VII deficiency
frequency?
homozygous symptoms?
what to suppliment?

Aquired

A

1/500,000
homozygous bleeding
Vit K suppliment

Aquired: vit K, oral anticoag, DIC, liver

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15
Q

X deficiency
heterozygous causes
homozygous causes

Aquired

special thing?

A

hetero mild bleeding
homo bleeding

Aquired: Vit K, oral anti, Liver, DIC

AMYLOIDOSIS - starch like in organs absorb X

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16
Q

XI deficiency
disease correlation?

high in what population

homozygous causes

special feature?

decreased…..what deficiency pathway

aquired?

A

hemophilia C
1/100,000 general population

high in jewish population

homozygous bleeding

ONLY CONTATC FACTOR THAT CAUSES BLEEDING

dec TAFI/ intrinsic cascade deficiency

DIC/LIVER

17
Q

XII deficiency
frequency?
what is increased?
causes? (because…)

aquired

A

common
recessive
APTT increased, no bleeding problems, exception

thrombosis problems (deficient activation of plasmin)

Aquired: DIC/ liver

18
Q

PK deficiency
frequency? what group?

what is increased

causes?

genetics?

A

RARE - in black families

APTT increased

no bleeding problems
thrombosis

auto dom and recessive

19
Q

HMWK deficiency
symptoms?
what is prolonged?
causes?

A

recessive
asympt
APPT prolonged
thrombosis

20
Q

XIII deficiency
common?
um
poor
bru

PT val
APTT val

what test?

A

Rare
umbilical stump bleeding
poor wound healing
bruises resolve slow/scars

PT normal
APTT Normal

Urea sol lysis

21
Q

DIC
acute…..in what

causes

consumption of

A

aquired

acute overwhelming of coag, 2nd fibrinolysis

bleeding and thrombosis
consumption of factors and plts

22
Q

Fibrinolysis lab testing

D DIMER
PLTS
Smear

A

D dimer increased/+
Plts decreased
smear: schistocytes

23
Q

Fibrinogenolysis lab

Ddimer
plts
smear

A

d dimer NEGATIVE
plts normal
smear normal

24
Q

Chronic DIC

seen in?
less….more…

speed?

A

aquired
compensated

seen in cirrhosis/metastatic cancer

less critical bleeding, more thrombosis

slowly, body can compensate better

25
Q

Liver disease
liver produces….

deficiency of…
impaired

accumulation of?

causes?

A

liver produces all factors, plasminogen, AT-III, alpha,2 antiplasmin

def of all factors/inhibitors/dysfunctional

impaired clearance

accumulation of plasminogen activators (DIC)

bleeding/thrombosis

26
Q

Renal disease
causes?

nephritic syndrom:

lose…

DIC causes?

A

bleeding/thrombosis

nephritic syndrom: uremic pts decrease plts, impaired

lose AT/protein C

DIC: renal damage

27
Q

Vit K deficiency

A

II, VII, IX, X
protein C

28
Q

Massive transfusion
loss of?

not usually a ….?

can cause what in what group? what do you give them?

T…

A

loss of labile factors V, VIII

not usually a bleeding problem

citrate toxicity - newborns/massives
give Ca++

TP: plts lose viability

(AA) Hemostasis (19 decks)

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