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Heme 2 > Bleeding, thrombotic, and Fibrolytic Disorders > Flashcards

Bleeding, thrombotic, and Fibrolytic Disorders Flashcards

1
Q

What are the platelet disorders?

A
  • immune thrombocytopenia
  • thrombotic thrombocytopenia
  • hemolytic uremic syndrome
  • henoch-schonelin purpura
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2
Q

If you see petechia and purpura what are you thinking the underlying cause is?

A

-PLATELETS!!!! its bleeding underneath the skin, these should not blanch.

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3
Q

Immune Thrombocytopenia (ITP):

  • etiology- primary/secondary
  • what is happening in ITP?
  • what age does this occur in?
A

Primary
- usually idiopathic

Secondary

  • autoimmune disorders (lupus)
  • medications (sulfonamides, thiazides, heparin)
  • viral infections (HIV, Hep C)
  • in children can be provoked by viral illness

Whats happening?
-pathologic abys bind to platelets resulting in accelerated platelet clearance.

-commonly occurs transiently in childhood, may be chronic in adults and more common in females.

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4
Q

Decreased platelets from immune response=???

A

-think thrombocytopenia!!!!!

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5
Q

ITP is an immune response in which…? (whats happening)

-quick and dirty explanation

A

-abys bind to platelets resulting in their premature destruction and inadequate production of platelets.

  • increased endogenous TPO clearance results in reduced levels of platelets.
  • megakaryocytes may be damaged by abys, making them less productive
  • suboptimal platelet production results from damaged megakaryocytes and reduced TPO levels

Quick and Dirty: ABY bind to platelet»> platelet destruction

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6
Q

Signs and symptoms of ITP

A
  • mucocutaneous bleeding (blood blister in mouth)
  • petechiae, purpura
  • spontaneous bruising
  • nosebleeds
  • gingival bleeding
  • retinal hemorrhage
  • excessive retinal bleeding
  • melena, hematuria
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7
Q

Dx of ITP includes the following…

A
  • thrombocytopenia
  • normal RBC morphology
  • prolonged bleeding time (this means its just a platelet problem)
  • +/- anemia
  • PT/PTT are normal
  • Bone marrow bx
  • -normal or increased number of megakaryocytes

*this is a dx of exclusion

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8
Q

Tx of ITP

A
  • treat w/ prednisone +/- IVIG(only give if no response to steroids within few days) for those w/ symptomatic bleeding or very low platelets.
  • splenectomy
  • Treat if platelet counts are less than 20-30,000or if significant bleeding
  • Prednisone oral: 2-10days
  • Dexamethasone: 4day/mo for 6mo.

-May give platelet transfusion if needed. (only give if you need to stop bleeding RIGHT NOW!)

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9
Q

Options for failure to respond to oral steroids:

A
  • IVIG or anti-D immune globulin
  • Rituximab (aby againts CD20)
  • thrombopoietin receptor agonist
  • splenectomy
  • BM transplant or chemo in severe case
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10
Q

Thrombotic Thrombocytopenic Purpura (TTP) ?

  • what is this?
  • quick and dirty explanation
A
  • disorder of inapproriate platelet aggregation leading to destruction of RBC and platelet consumption
  • make abys against ADAMTS-13 which is responsible for cleaving large vWF molecules into smaller pieces.

Large vonwillibrans molecules are pro-thrombotic. If we dont have ADAMTS-13 cleaving this into smaller pieces we will clot easier and more often (inappropriately)

*emboli shear force on RBC leads to destruction (in terminal vessels and get lodged in kidney and brain)

Quick and Dirty: ABY bind to ADAMST-13»large vWF»clot»RBC destruction and platelet consumption

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11
Q

What is TTP characterized by?

A
  • thrombocytopenia
  • hemolytic anemia
  • inapprorpiate platelet aggregation and formation of fibrin.
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12
Q

Primary and Secondary Causes of TTP?

A
  • Primmary: autoimmune
  • Secondary:
  • -cancer
  • -BM tx
  • -Pregnancy
  • -Meds: acyclovir, clopidogrel
  • HIV
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13
Q

What are the 5 main characteristics of TTP?

Which are required for dx? (these will be starred)

A
  • Thrombocytopenia*
  • Microangiopathic hemolytic anemia*
  • Neurologic sx
  • kidney failure (dont have to have this but may show up later in disease course)
  • fever
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14
Q

Sx of TTP?

A

Sx caused by secondary effects of underlying microvascular clotting disorder:

febrile

pallor

malaise

diarrhea

thrombocytopenia = bleeding, bruising

petechiae, purpura

-microvascular clotting leading to organ damage= kidney failure (high creatinine, high BUN, low urine output, edema, blood in urine(RBC casts)),

neurologic sx (HA, diff speech, seizure, transient paralysis, coma, confusion)

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15
Q

What will you see in peripheral blood smear of microangiopathic hemolytic anemia?

A

-schistocytes (helmet cells) and low platelets

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16
Q

What are some lab abnormalities seen in TTP?

A
  • elevated indirect bilrubin
  • decreased serum haptoglobin ( free heme binds to haptoglobin; this is why its low)
  • microangiopathic hemolytic anemia
  • severely elevated LDH
  • thrombocytopenia
  • marked anemia
  • reticulocytosis
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17
Q

Thrombocytopenic purpura Tx

A

-plasma exchange (plasmapheresis)

*** do NOT give platelet transfusion, they ‘FUEL THE FIRE’

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18
Q

Hemolytic Uremic Syndrome Causes

A

Most commonly it is secondary to -E. Coli 0157:H7

But may be caused by,

  • Streptococcus pneumonia
  • shigella dysenteriae*
  • chemo drugs
  • anti-platelet medications
  • HIV
  • pregnancy
  • genetics
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19
Q

HUS pathophysiology

A

HUS is caused by endothelial damage secondary to bacterial toxins:

-endothelial damage leads to:
–leukocyte activiation
–platelet activation
–inflammation
–multiple thromboses
resulting in RBC destruction d/c shear forces in the vessel leading to hemolytic anemia.

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20
Q

What are the three (TRIAD) things you need to have to be dx w/ HUS?

A
  • microangiopathic hemolytic anemia
  • acute kidney injury and renal failure
  • thrombocytopenia (platelets low becaus they are being used up and cannot be replaced fast enough)
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21
Q

What is the main difference between TTP andd HUS?

A

-both have hemolytic anemia but in HUS the kidneys are non-functional and TTP has more neurological sx.

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22
Q

HUS signs and symptoms

A
  • recent or current bloody diarrhea
  • abd pain
  • decreased urine output
  • hematuria
  • renal failure
  • hypertension
  • neurologic changes
  • edema

*same as TTP but with RENAL FAILURE!

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23
Q

Tx of HUS?

A
  • general tx is supportive
  • transfuse RBC and platelets
  • dialysis of symptomatic uremia
  • nutritional and electrolyte support
  • if thought to be secondary to an autoimmune process may be consider plasma exchange.
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24
Q

Four major sx associate with Honoch-Schonelin Purpura (HSP)

A
  • palpable purpura
  • athritis/arthralgias
  • abd pain
  • renal disease
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25
Q

HSP
-cause?
-what age group is affected?
-

A
  • just a vasculititis, no probs bleeding or platelets, triggered by streptococcal upper resp. infection.
  • 90% of cases in 3-15years
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26
Q

Tx of HSP?

A
  • treatment is supportive
  • NSAIDS or glulcocorticoids
  • usually just clears but you need to continue to monitor them for renal failure from IgA deposition.
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27
Q

HSP work up includes..?

A
  • biopsy of skin lesions*
  • CBC
  • CMP
  • urinalysis (may show hematuria +/- rbc casts (clots formed in rental tubule being passed out of body)
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28
Q

What are the bleeding disorders?

A
  • Hemophilia
  • VonWillebrands Disease
  • Disseminated intravascular coagulation
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29
Q

How do you get hemophilia?

What is the most common type of Hemophilia?

A
  • its genetic, x-linked recessive trait in men. women may be carriers
  • Hemophilia A
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30
Q

Deficiency of what factor will result in hemophilia A? Hemophilia B? Hemophilia C?

A
  • Factor VIII deficiency
  • Factor IX deficiency (Christmas disease)
  • Factor XI deficiency
31
Q

Characteristic bleeding in hemophilia?

A

spontaneous hemarthrosis, but other common sites of bleeding are:
Joints: knees, ankles, elbows
Skin, muscle
GU, GI

32
Q

What is hemophilia?

A

deficiency of coagulation factors, these differ between types A (VII), B (IX), and C(XI).

33
Q

Tx of Hemophilia?

A

-lifelong replacement of deficient clotting factor, usually 3x/week.

  • avoid aspirin
  • cryoprecipitate
  • DDAVP (stimulates vonwillebrands factor)
34
Q

What do you need for diagnosis?

A
  • PTT is prolonged
  • PT, bleeding time, platelets, vonWillebrand factor are normal.
  • CONFIRMED by decreased levels of VIII, IX, or XI
35
Q

Do Hemophiliacs bleed longer, bleed more quickly, or have a shortened lifespan?

A

-Bleed longer.

36
Q

Functions of vonWillebrand factor?

A
  • binds platelets to form the initial platelet plug

- binds w/ factor VIII to prolong its half-life.

37
Q

How do you get vonWillebrands disease?

A

-genetics! its autosomal dominant affecting both sexes equally.

38
Q

What is the most common INHERITED bleeding disorder?

A

-vonWillebrands type 1.

39
Q

What are the types of vonWillebrand disease?

A

-Type 1: quantitatiev abnormality of vWF, vWF functions normally they just dont have enough.

Type 2: qualitative abnormality, have enough vWF, its just NOT functional. Doesnt bind the way its supposed to.

Type 3: rare, undetectable levels of vWF and severe bleeding in infancy and childhood.

40
Q

Sx of vonWIllebrands diseaes

A
  • easy bruising
  • skin bleeding
  • prolonged bleeding from mucosal surfaces
  • asymptomatic
41
Q

dx of vonWillebrands clues from personal and family hx

A
  • nosebleeds >10mins in childhood
  • lifelong easy bruising
  • bleeding following dental extractions/surgery
  • heavy menstrual bleeding or post partum
42
Q

Lab findings in vonWillebrands

A

-Plasma vWF antigen (serum concentration of vWF)

  • Plasma vWF activity determined by:
  • -vWF:Rco and vWF collagen

-Factor VIII activity

  • Normal PT
  • PTT normal or prolonged depending on the factor VIII activity
43
Q

Tx of vWF:
Type 1
Type 2
Type 3

A

Minor Major
1: DDVAP, DDAVP vWF conc.

  1. DDAVP vWf conc., vWF conc
  2. vWF conc., vWF conc.
44
Q

Why dont you give DDAVP to pts w/ Type 3 vonwillebrands factor?

A

-you dont give DDAVP because they dont make any vWF. its purpose is to activate vWF but they dont make any vWF so it doesnt do any good.

45
Q

What is Disseminated Intravascular Coagulation? Explain what happens in “living room language”

A

simultaneous hemorrhage and thrombosis at the same time.

What happens:

  • massive release of tissue factor stimulated by injury.
  • tissue factor set the coagulation system in place
  • clotting occurs
  • clotting factors and inhibitors are all consumed
  • clots further trap circulating platelets leading to ischemia
  • **at the same time:
  • excess thrombin activates plasmin resulting in fibrinolysis. The breakdown of clots= fibrin degredation products which have further anticoagulant properties

*plasmin also activates the complement and kinin system=shock

46
Q

Causes of DIC

A
  • Cancer
  • Obstetric Complications
  • Sepsis
  • Massive tissue injury (trauma, burns, hyperthermia)
  • snake bite
47
Q

Where do all of the clots lodge in DIC?

A

-in the periphery, commone to have ischemia in handds and feet, capillaries of the skin.

May see petechia, purpura, gangrene, renal and liver failure

48
Q

Laboratory findings of DIC

A
  • thrombocytopenia
  • prolonged PT and PTT
  • low fibrinogen (b/c its all being used up)
  • increased levels of D-dimer (fibrinogen degredation products)
  • Schistocytes (helmet cell)
  • low levels of ATIII, Protein C and S, factors V, VIII, X, XIII
49
Q

Treatment of DIC

A

tx is targeted at underlying cause.
ex. sepsis- treat the infection

  • anticoagulants only to prevent imminent death
  • platelets or coagulation factor replacement in the case of platelets 100mg/dL if significantly elevated PT/INR
50
Q

What things stimulate clottting?

A
  • endothelial damage and inflammation
  • -atherosclerosis
  • -DM
  • -Tobacco use
  • -cancers

-elevated platelet levels

51
Q

Conditions and risk factors that accelerate the activity of the clotting system?

A
  • Pregnancy
  • oral contraceptives
  • postsurgical state
  • malignancy
  • hereditary clotting disorders

Risk factors:
-stasis/immobility

  • low cardiac output (CHF)
  • obesity (extra body fat has increased estrogen levels, estrogen levels increase coagulation)
  • sleep apnea
52
Q

What are the hereditary clotting disorders?

A
  • Protein C
  • Protein S
  • Antithrombin III
  • Factor V leiden
  • Anti-phospholipid aby
53
Q

How does protein C deficiency affect hemostasis?

A

-Protein C inactivates Factor V and VIII thereby inhibiting coagulation, in the event of DEFICIENCY, the actions of factor V and VIII lead to excessive clotting

54
Q

Signs and symptoms of Protein C deficiency

A
  • usually no signs or sx are recongnized until clots form.
  • -thrombosis
  • -deep vein thrombosis
  • -PE
  • -Thrombophlebitis
55
Q

What tests do you order when working up Protein C deficiency?

A
  • Protein C
  • PTT, PT
  • Thrombin time
  • Bleeding TIme
  • Medical and family hx may be revealing of hx of thromboebolism
56
Q

-Tx of Protein C deficiency

A
  • treat w/ anticoagulants if high risk for clotting (surgery/hospitalization)
  • Chronic anticoagulation if hx of thrombosis (only do this if they have hx of clots)
57
Q

Protein S function?

A

-supports the function of protein C, its needed fro proper function of Protein C.

58
Q

How does Protein S deficiency affect hemostasis?

A

-a deficiency in protein S results in diminished ability of protein C to inactivate factors V and VIII resulting in excessive clotting.

59
Q

Symptoms, Dx, and tx of Protein S deficiency

A

-these are all the same as protein C deficiency.

Sx:

  • usually no signs or sx are recognized until clots form.
  • -thrombosis
  • -deep vein thrombosis
  • -PE
  • -Thrombophlebitis

Tx:
-treat w/ anticoagulants if high risk for clotting (surgery/hospitalization)

-Chronic anticoagulation if hx of thrombosis (only do this if they have hx of clots)

60
Q

How do you get antithrombin III deficiency? At what ages is this disorder most common?

A
  • hereditary disorder, autosomal dominant

- 15-35years

61
Q

What are the symptoms of Anti-thrombin III deficiency?

A
  • recurrent venous thrombosis*
  • Pulmonary embolism
  • repetitive intrauterine fetal death
62
Q

Function of ATIII?

A

inhibitor of thrombin, factors X and IX

63
Q

How does ATIII deficiency affect hemostasis?

A

-leads to tendency to form clots

64
Q

Work up if suspected ATIII deficiency? Tx?

A
  • Antithrombin-heparin cofactor assay (measures functional AT activity) is the best screening test for AT deficiency
  • if pt on anticoagulatn or in midst of thrombotic crisis the test results can be skewed.
  • PT and PTT should be normal.
  • Same as Protein C and S deficiency
65
Q

What is the most common genetic disorder to cause DVT?

A

Factor V Leiden, normal amount of factor V but its mutated so it doesnt function normally

66
Q

How does Factor V Leiden deficiency affect hemostasis?

A
  • blood has increased tendency to clot and is most likely to occur in the veins.
  • Lack of Factor V Leiden decreases the anticoagulant activity of the activated protein C.
67
Q

Sx of Factor V Leiden deficiency?

A
  • asymptomatic until clot forms resulting in ….
  • Thrombophlebitis
  • deep vein thrombosis
  • Pulmonary embolism
68
Q

Factor V Leiden deficiency Tx

A
  • chronic coagulation if presenting w/ blood clots

- if no hx of thrombosis then only use anticoagulants if at risk for clots d/t hospitalization or surgery

69
Q

what is Antiphopholipid Aby syndrome?

How does this affect hemostasis?

A
  • clotting disorder secondary to autoimmune process.
  • autoimmune hypercoaguable state caused by antiphospholipid abys
  • abys lead to arterial and venous clot formation
70
Q

Complications of Antiphopholipid Aby syndrome?

A

-pregnancy: miscarriage, stillbirth, preterm delivery, severe eclampsia

end organ damage

71
Q

What are the types of Antiphopholipid Aby?

A
  • Lupus anti-coagulant
  • Anti-cardiolipin aby
  • Anti-beta2-glycoprotein
72
Q

What are the primary and secondary causes of Antiphopholipid Aby syndrome?

A

Primary: no other related disease to cause abnormal abys

-other autoimmune disorders such as lupus

73
Q

Dx of Antiphopholipid Aby Syndrome

A

Must have one of the abnormal bays PLUS hx of clotting

  • anti-cardiolipi abys IgG or IgM
  • lupus anticoagulant
  • anti-beta2glycoprotein I IgG and/or IgM
74
Q

Tx of Antiphopholipid Aby Syndrome

A

-chronic anticoagulation and antiplatelet therapy:
Aspirin

Warfarin

Heme 2 (8 decks)

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