"Factor bleeding" - deep joint bleeding - prolonged bleeding after dental work \ rarely, mucosal hemorrhage

Bleeding Disorders--Krafts Flashcards by Robbie Gruhl

Platelet bleeding is characterized by…

spontaneous bleeding

petichaie

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Factor bleeding is characterized by…

big bleeds after trauma

esp. into deep joints

*lots of bleeding, not like petichae (like platelet bleeding)

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Petichiae are characteristic of…

platelet bleeding

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Big, joint space bleeding is characteristic of…

factor bleeding

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Type 1 vWD problem

decreased vWF

(75%)

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Type 2 vWD problem…

abnormal vWF

(25%)

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Type 3 vWD problem…

no vWF

(5%)

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Sx of vWD

mucosal bleeding

deep joint bleeding in severe cases

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vWD

Bleeding time

PTT

INR

[vWF]

Platelet aggregation studies

Bleeding time: prolonged

PTT: prolonged “corrects” w/ mixing study

INR: normal

[vWF]: normal, except type 2

Platelet aggregation studies: abnormal

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vWF binds which GP?

GPIb

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GPIa

GPIb

GPIIb/IIIa

GPIa: collagen

GPIb: vWF

GPIIb/IIIa: fibrinogen

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Tx of vWD

cryoprecipitate (contains vWF and VIII)

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Hemophilia inheritance pattern

X-linked recessive

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Most common factor deficiency

Hemophilia A

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Hemophilia A deficiency

Factor VIII

(levels decreased)

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Hemophilia A Sx

“Factor bleeding”

deep joint bleeding
prolonged bleeding after dental work

*rarely, mucosal hemorrhage

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Hemophilia A

INR, TT, platelet count, bleeding time

PTT

Factor VIII assays: abnormal

DNA studies: abnormal

INR, TT, platelet count, bleeding time: normal

PTT: prolonged (corrects with mixing study)

Factor VIII assays: abnormal

DNA studies: abnormal

Tx of Hemophilia A

Factor VIII replacement

Hemophilia B pathology

Factor IX deficiency

Bernard-Soulier Syndrome

pathology

abnormal Ib (vWF receptor)

Bernard-Soulier Syndrome

severe bleeding

big platelets

Glanzmann Thrombasthenia

pathology

GPIIb/IIIa deficiency

no aggregation

Gray platelet syndrome

pathology

no alpha granules

mild bleeding

Delta granule deficiency

pathology

absent delta granules

may be part of another syndrome

Why do you bleed in DIC?

use up all platelets use all clotting factors

What type of anemia is associated with DIC?

microangiopathic hemolytic anemia

What sets off DIC?

initiation of coagulation | (it's not a platelet problem)

Big causes of DIC

malignancy OB complications sepsis trauma **MOST** cases acrynym

**_DIC_** INR, PTT, TT FDPs Fibrinogen

_INR, PTT, TT_: prolonged _FDPs_: increased _Fibrinogen_: decreased

Idiopathic Thrombocytopenic Purpura pathology

antiplatelet antibodies GPIIb/IIIa or Ib splenic macrophages eat platelets

Chronic vs Acute Idiopathic Thrombocytopenic Purpura

_Chronic_: big problem--bleeding into brain _Acute_: abrupt, follows viral illness (usually children)

Labs in idiopathic thrombocytopenic purpura

thrombocytopenia many/bigger megas in BM bigger platelets in smear

TTP pathology

ADAMTS13 deficiency ADAMTS13 normally cleaves new, large vWF to less active bits if they are not able to be cleaved, then you get a lot of platelet aggregation --\> little clots everywhere

TTP pentad

MAHA thrombocytopenia fever neurologic defects renal failure

Why do you get neurlogic defects in TTP?

many little clots that end up going to the brain

Two things you will have in TTP

microangiopathic hemolytic anemia thrombocytopenia + neurological deficits

TTP Tx

_acquired_: plasmapheresis _hereditary_: plasma infusions

Hemolytic Uremic Syndrome pathology

E. coli infections - most likely form toxin - endothelial damage (platelet activation) \*this is the reason that E. coli outbreak patients are hospitalized

Hemolytic Uremic Syndrome clinical findings

blood diarrhea then renal failure 5% fatality rate

Tx of hemolytic uremic syndrome

NOT ABX!! supportive care dialysis