coag diseases/ anticoagulation therapy

inherited factor deficiencies

VWF

Hemophilia A

Hemophilia B

Hemophilia C

Dysfibrinogenemia

Factor 13

VWF

Dec GPIb problem= no adhesion

Type 1: low VWF

Type 2: qualitative

Type 3: NONE

Increase PFA, increase PTT (cuz factor 8)

normal PT

No aggregation to ristocetin

Hemophilia A

Factor 8 deficiency

Increased PTT

Hemophilia B

Factor 9 deficiency

Increased PTT

Dysfibrinogenemia

Dys = dysfunctional fibrinogen but normal amount

Increased PT and PTT,

Hemophilia C

Factor 11 deficiency

ASHKENAZI JEWS

Factor 13 deficiency

Delayed bleeding***

Will form clot (no bleeding) but no cross link from 13 (delayed bleeding)

5M urea test (if clot dissolved= factor 13 deficient)

inhibitors

When mixing study does not correct

Supplement with DDAVP

Platelet disorders

Fanconi anemia

May hegglin

Scott syndrome

Hypoplasia (neonatal or acquired)

ITP (immune thrombocytopenic purpura):

Drug induced ITP

HIT (heparin induced thrombocytopenia)

Nonimmune thrombocytopenia:

Pregnancy

HELLP

TTP (thrombotic thrombocytopenic purpura)

HUS

DIC

Fanconi anemia

Bone marrow problem

Decrease plts

May hegglin

PLT maturation problem

Large platelets, but normal function

Less platelets

Dohle like bodies

Scott syndrome

Decrease phospholipid activity = no clots

Hypoplasia

Neonatal: Decreased megakaryos and plts

Acquired: toxin exposure decreases plts

ITP

Decreased plt number

Large platelets

Caused by immune system ab

Drug induced ITP

Ab to drug reacts with plt

HIT

Ab to heparin-PF4

PTP

Post transfusion purpura

Ab to HPA-1a

Nonimmune thrombocytopenia

Pregnancy

HELLP

TTP

HUS

DIC

Pregnancy

Slight decrease in plts from hypercoagulable state

Returns to normal post delivery

HELLP

Hemolysis Elevated Liver LD Platelet decrease

High AST ALT LD

Low plt

Hemolysis and preeclampsia (protein in urine)

TTP

Decrease in ADAMTS13, can’t break down VWmultimers

Causes MAHA (microangiopathic hemolytic anemia)

Inc unconj bili and dec in hapto from hemolysis

HUS

In children with E. coli/shigella infections

Toxins attack gloremulus

Bernard soulier

Adhesion problem cuz dec GPIb

GIANT plts, decreased plts

Epi, ADP, collagen: NORMAL

ristocetin: ABNORMAL

Glanzmanns thrombasthenia

Aggregation problem cuz dec GPIIb

Epi, ADP, collagen: ABNORMAL

ristocetin: NORMAL

Storage pool (dec in granules)

Chediak Higashi

Wiskott Aldrich

Chediak higashi

Decrease in delta granules→ dysfunction

Also affects WBCs

Epi, ADP: abnormal in 2nd wave

Wiskott Aldrich

Dec IgM/IgG

Inc IgA/IgE

Epi, ADP, collagen: decreased

Grey plt syndrome

Dec in alpha granules

Hermansky pudlak

Dec in dense granules

PLT agonists

Epinephrine: aggregation 2,

Collagen: adhesion 1

ADP: plt shape change, aggregation 2

ristocetin: sens to VWF and GPIb

unfractionated heparin

Inhibit 12, 11, 10, 9 (affect PTT)

Low molecular heparin

Inhibits 11, 10 (affects PTT)

Warfarin/Coumadin

“War against K”

Inhibits 10, 9, 7, 2 (affects PT cuz of 7)

Apixaban

Think “ban Xa”

Direct Xa inhibitor

DTI/pradaxa

Direct thrombin inhibitors

Aspirin

Anti-platelet drug

Inhibits cyclooxygenase, so no plt granule secretion

Plavix

Targets ADP P2y12 receptor

Less plt aggregation for GPIIb

Thrombin time

Conversion of fibrinogen to fibrin

Not a measure of PT or PTT

Affected by heparin (falsely increases)

Reptilase time

Same test as Thrombin Time

But is not affected by heparin