Term
| Types of platelet disorders: |
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Definition
Idiopathic Thrombocytopenic Purpura (ITP)
Heparin Induced Thrombocytopenia
Thrombotic Thrombocytopenia Purpura (TTP)
Hemolytic Uremic Syndrome |
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Term
| Types of coagulation disorders |
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Definition
von Willebrand's disease
Hemophilia A
Hemophilia B
Vitamin K deficiency
Disseminated Intravascular Coagulation (DIC)
Coagulopathy of Liver Disease
Factor XI Dificiency |
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Term
| Types of bleeding in Platelet Disorders |
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Definition
1. Mucous Membrane
2. Epistaxis - nose bleeding
3. Petechiae - blood escaping from blood vessels; they do NOT blanch
4. Menorrhagia - heavy menses |
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Term
| Types of bleeding in Coagulation Disorders |
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Definition
Bruising
Soft tissue
Muscles
Joints |
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Term
Immune Thrombocytopenic Purpura?
Definition?
Common in which age?
Etiology?
SSx?
Diagnostic test?
Prognosis?
Treatment? |
|
Definition
- Autoimmune disorder in which an IgG autoantibody binds to platelets and destroys them
- Acute: 80% children (2-7) and 20% adults Chronic: 20% kids, 80% adults
- Acute: precipitated by viral infection
Chronic: in adults with collagen vascular Dz or lymphocytic leukemia
- kids: petechiae and purpura
adults: bruising, epistaxis, and menses
- Platelet <10,000; test of exclusion; enlarged platelets, nml RBC size
- Good for remission
- Prednisone; IVIG |
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Term
Thombotic Thrombocytopenia Purpura
Definition?
Common in which age?
Etiology?
SSx?
Diagnostic test?
Prognosis?
Treatment? |
|
Definition
- thrombosis in terminal arterioles and capillaries
- young adults 20-50
- Endothelial cell damage causes incr vWF release that induces platelet aggregation
- anemia, neurologic changes, azotemia (incr blood urea nitrogen),fever,low plt
- SCHISTOCYTE, low plts, rising creatinine
- 80-90% recover completely; 20% relapse
- Steroids; plamapharesis |
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Term
Hemolytic Uremic Syndrome?
Definition?
Common in which age?
Etiology?
SSx?
Diagnostic test?
Prognosis?
Treatment? |
|
Definition
- microangiopathy
- usually in children, due to diarrheal illness (secondary to shigella, salmonella, E. coli);
in adults: often by estrogen or pospartum
- Renal failure, anemia, thrombocytopenia, (no neurological manifestations)
- incr RBC fragmentation; incr LDH
- children: supportive care; adults: plasmapharesis with FF replacement |
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Term
| Which platelet disorder shows up after a diarrheal episode secondary to infection by E.coli, salmonella, or shingles? |
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Definition
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Term
| which disorder shows up after a viral infection? |
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Definition
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Term
| Schistocytes are seen in which disorders? |
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Definition
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Term
| disorder due to the release of increase vWF that induces platelet aggregation |
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Definition
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Term
| Disorder that has a plt count < 10,000 and enlarged platelet; nml RBC morphology |
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Definition
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Term
| Disorder that has a plt count < 10,000 and enlarged platelet; nml RBC morphology |
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Definition
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Term
| Which drug is most common for inducing thrombocytopenia? |
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Definition
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Term
Heparin-induced thrombocytopenia
Definition?
Common in which age?
Etiology?
SSx?
Diagnostic test?
Prognosis?
Treatment? |
|
Definition
-most common drug to induce thrombocytopen
- most common cause of thrombocyt. in hospitalized pts
- Autoantibody IgG interacts with Platelet Factor 4 on platelet --> thombosis
- Highest risk after 4 days
- cause: self-induced and self-limiting
- arterial or venous thrombus
-test with HIT antibodies
- thrombin inhibitors; stop heparin; use LMWH or coumadin |
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Term
von Willebrand Disease?
Definition?
Epidemiology?
Etiology?
SSx?
Different types!!
diagnostics?
Prognosis?
Treatment? |
|
Definition
- Autosomal dominant condition
- Have defective vWF with low Factor VIII levels
- 1% of the population
- Platelet dysfunction and deficient Factor VIII
- mucosal bleeding, epistaxis, superficial (petechiae), menorrhagia
- low levels of VIII:c, VWFAg, and RCof
- excellent
- FFP and cryoprecipitate |
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Term
|
Definition
- mild/most common (>80%)
- Reduced quantity of VWF
- VWF nml, just reduced
- labs: VIII:C, VWF:Ag, and RCof are all partially decreased in parallel levels |
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Term
|
Definition
- mild to moderate bleeding; 15%
- Qualitative defects in function
Type 2A: lacks HMW multimers
Type 2B: qualitative variants/platelet binding
- VIII:C, VWF:Ag, and RCof are decreased to different degrees (= qualitative variants) |
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Term
|
Definition
- Severe bleeding (<5%)
- VWF very low or absent
- VIII:C, VWF:Ag (ex. <12), + RCof: all really low |
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Term
|
Definition
FFP and cyroprecipitate
* avoid aspirin |
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Term
Hemophilia A
Definition?
Epidemiology?
Etiology?
SSx of bleed?
Different types!!
diagnostics?
Complications?
Treatment? |
|
Definition
- Deficiency of clotting factor VIII
- Mostly males;
80% of clotting deficiencies (hemophilias)
- X-linked recessive; hereditary disorder
- pain, swelling, and decr ROM to joint
- aPTT prolonged, PT nml; 1:1 mix nml; Factor VIII assays
- hemophilia inhibitors
- recombinant factor VIII assay |
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Term
Hemophilia B
Definition?
Epidemiology?
Etiology?
SSx of bleed?
Different types!!
diagnostics?
Complications?
Treatment? |
|
Definition
-Deficiency of clotting factor IX --> bleeding diathesis
- Mostly males;20% of clotting deficiencies (hemophilias)
- X-linked recessive; hereditary disorder
- pain, swelling, and decr ROM to joint
- aPTT prolonged, PT nml; 1:1 mix nml; Factor VIII assays
- hemophilia inhibitors
- recombinant factor IX assay |
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Term
Define Mild hemophilia
How much of Factor VIII or IX do they have? |
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Definition
no spontaneous bleeding; may have major hemorrhage after surgery or trauma
6-50% of factor level |
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Term
Define Moderate hemophilia
How much of Factor VIII or IX do they have? |
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Definition
- mod amt of bleeding,
- 1-5% of Factor level |
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Term
Define Severe hemophilia
How much of Factor VIII or IX do they have? |
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Definition
- Spontaneous hemorrhage into tissue and joints; if left untreated --> arthropathy and joint destruction
- <1% of factor level |
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Term
| What is the mot common inherited bleeding disorder? |
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Definition
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Term
| Vitamin K is required for the synthesis of which Factors? |
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Definition
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Term
Vitamin K deficiency
Etiology?
SSx?
Diagnostics?
Prognosis?
Treatment? |
|
Definition
-dietary reduced intake, antibiotics (reduces absorption), and hemorrhagic disease of the newborn
- no specific clinical features; bleeding can occur at any site
- aPTT and PT are increased; nml BT
- excellent
- Sub-Q vit K |
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Term
Factor XI Deficiency
Definition?
Epidemiology?
Etiology?
SSx?
Diagnostics?
Treatment? |
|
Definition
Hemophilia C
- Deficiency of clotting factor XI
- Ashkenazi jews; predominantly males;
- Autosomal recessive; family history
- may not bleed, only severe forms
- PTT prolonged; Factor XI assay
- Factor XI with FFP; avoid aspirin |
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Term
Disseminated Intravascular Coagulation (DIC)
Definition?
Epidemiology?
Etiology?
SSx?
Acute and Chronic
Diagnostics?
Prognosis?
Treatment? |
|
Definition
- simultaneous hemolysis and hemostasis
- common disease in the hospital pop.
- Deposition of fibrin in small blood vessels --> thrombosis + end-organ damage
Depletion of clotting factors & platelets --> bleeding diathesis
- Triggered by sign tissue injury; Consumptive process
- acute and chronic types
- PT, PTT,TT, Platelet, FDPs+D-dimer, clotting factor,
- life threatening
- treatment of underlying illness |
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Term
Coagulatory of liver disease?
Definition?
Diagnostics?
Prognosis?
Treatment? |
|
Definition
- liver is the site of synthesis of all the coagulation factors except factor VIII
- Shows abnml PT faster because VII has a short half-life
- Vitamin K-dependent factors (2,7,9,10) are first to be affected
- depends on underlying liver disease
- FFP; platelet transfusion |
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Term
Acute DIC
PT
aPTT
TT
Platelet
FDPs + D-dimer
Clotting factor |
|
Definition
- Increase
- Increase
- Increase
- Decrease
- Increase
- Decrease |
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Term
Chronic DIC
PT
aPTT
TT
Platelet
FDPs + D-dimer
Clotting factor |
|
Definition
- Increase
- Increase
- Increase
- Normal
- Increase
- Normal |
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