2/12 - Bleeding Disorders

Front 1

Large bruises called?

Back 1

ecchymoses

Front 2

Why does cirrhosis cause bleeding?

Back 2

b/c coagulation factors are made in the liver (low fibrinogen w/ cirrhosis)

Front 3

Bleeding into soft tissue called?

Back 3

Hematoma

...classically cause by hemophilia

Front 4

Pinpoint hemorrhages called?

A little larger version of them called?

Back 4

petechiae

purpura

...both associated with platelets

Front 5

hematochezia?

melena?

Back 5

Hematochezia: lower GI bleed; bright red blood in stool (often due to hemorrhoids)

Melena: upper GI bleed; dark, tarry stool

Front 6

T/F Most patients with bleeding disorders do not have problems in the blood

Back 6

T

-usually do NOT requre blood tests

Front 7

Clinical Presentation's of what?:

Hematemesis?

Menorrhagia?

Melena?

Hematuria?

Back 7

Hematemesis - portal hypertension & cirrhosis

Menorrhagia - aspirin/NSAID overuse

Melena - Colon cancer

Hematuria - renal calculus

Front 8

Primary hemostasis problme will result in what kind of bleeding?

Back 8

Mucocutaneous bleeding:

Petechiae, purpura, menorrhagia, mucosal bleeds

Front 9

Controling components of primary hemostasis are?

Back 9

Vessel wall
Platelets
vW factor

Front 10

Two signs of scurvy?

Back 10

1) Vascular abnormalities - bleeding deficiency (ie gums & legs)

2) corkscrew hairs

Front 11

Congenital causes of vascular abnormalities?

Back 11

1) Hereditary hemorrhagic telangiectasia

2) CTD (ie Marfan's)

...all vascular abnormalities manifest as petechial/purpuric hemorrhages

Front 12

1st thing that happens in primary hemostasis? Describe

Back 12

Platelets adhere to hte subendothelium

vWF bridges the platelets with the subendothelium

Platelets then become more active and spill granular contents that recruit more platelets (secondary hemostasis)

Front 13

Not having enough platelets called?

Back 13

Thrombocytopenia

-the MC cause of bleeding!

Front 14

Dysfunctional platelets may be caused by?

Back 14

aspirin

Front 15

substrate that bridges platelets to other platelets

Back 15

Fibrinogen (substrate for Fibrin - secondary hemostasis)

Front 16

Three main causes of platelet disorders?

Back 16

1)Thrombocytopenia (not enough platelets)

2)problem with collagen or vWF

3)Cirrhosis (low fibrinogen)

Front 17

CBC tells you what information?

Back 17

Complete Blood Count

Platelet count and morphology

Front 18

MPV means what?

Back 18

Mean platelet volume - SIZE of platelets

Front 19

Causes of over-destruction of platelets desprite normal produection by bone marrow (4)

Back 19

1) Antibodies against platelets (immune thrombocytopenic purpura, ITP

2)DIC

3)sepsis

4))hypersplenism (as in cirrhosis)

Front 20

When platelets are low b/c of increased platelet destruction, the bone marrow is making ____ platelets to compensate for the loss

Back 20

LARGER platelets (ie in ITP or DIC)

Front 21

When platelets are low b/c of decreased platelet production, the bone marrow is making ____ platelets to compensate for the loss

Back 21

normal-sized in

Front 22

Causes of inhibition of Bone marrow platelet production

Back 22

1) Drugs

2) Toxins

3) Tumors

4) Acute leukimic process (no room for megakaryocytes to grow)

Front 23

T/F The reference range for platelet counts are quite large?

Back 23

T

150000-440000 per microliter of blood

Front 24

Bone marrow may increase platelet production by up to ___ from baseline?

Back 24

six-fold

Front 25

Average platelet life span?

Back 25

8-12days

Front 26

Percentage of platelet pool in the spleen?

Back 26

33% (1/3!)

those with cirrhosis, portal hypertension and large spleens have thrombocytopenia

Front 27

Name and describe problem of the 2 congenital platelet disorders

Back 27

Bernard Soulier Syndrome (BSS) - deficiency of Gp1b receptor, which binds the vWF for platelet adhesion to endothelium

2)Glanzman's thrombasthenia - defect of the GpIIb-IIIa complex, which is the fibrinogen receptor


"Think that B comes before G and GpIb comes before GpIIb-IIIa"

Front 28

BSS has similar manifestation to what other disease?

Back 28

vWF disease, much more common than BSS

Front 29

If you see thrombocytopenia AND giant platelets think

Back 29

BSS

Front 30

Mild QUANTITATIVE abnormlaity of vWF leading to mucotaneous bleeding

Back 30

Type 1 vWF disease

Front 31

Mild QUALITATIVE abnormlaity of vWF

Very severe deficiency of vWF?

Back 31

Type 2 vWF disease

Type 3 vWF disease

Front 32

T/F vWF disease affects male and females equally?

Hemophilia?

Back 32

T

F, hemophilia is mostly X-linked

Front 33

One problem with making the diagnosis of vWF disease (which requires blood testing) is what?

Back 33

vWF FLUCTUATES b/c it is one of the acute phase reactants (like fibrinogen and Factor VIII)

Front 34

Disorders of secondary hemostasis (coaagulation cascade) usually result in?

Problem in Adults? Children?

Back 34

Hemarthrosis (soft tissue or joint bleeds)

Adults = acqurired (drugs, diseases or diet)

Children = congenital, even in absence of family history

Front 35

If fibrinogen is below ___ mg/dL, the patient will likely bleed

Back 35

50 mg/dL

Front 36

"Match that starts the fire" of clotting cascade?

Activates what?

Back 36

Factor vii and Tissue Factor (TF)

-fairly short lived

-activates Factors IX and X

Front 37

"Fuel fo rthe flame" of the clotting cascade?

Back 37

Factors X and IX

Front 38

What transforms fibrinogen into fibrin

Back 38

Thrombin

Front 39

Most common/important acquired causes of secondary hemostasis disorders (2)

Back 39

1) Liver disease (Cirrhosis - Hep C most commmon cause of in USA)

2) Oral anticoagulation (ie warfain

Front 40

Risk of bleeding with warfarin increases with what concomitant problems (3)?

Back 40

1) A ^ INR (international normalized ratio)

2) A history of stroke or GI bleed

3) Age > 65

Front 41

clotting so much that you use up the platelets and coagulation factors called?

treatment?

Back 41

DIC

treat by addressing underlying cause

Front 42

Most important genetic disorder of the coagulation cascade?

Prevance?

Back 42

Hemophilia A & B

A 5 times more comon than B, but CLINICALLY INDISTINGUISHABLE

Front 43

Factor deficiency of Hemophilia A? B?

Back 43

Hemophilia A = Factor VIII deficiency

Hemophilia B = Factor IX deficiency