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Hemostasis and thrombosis is dependent on 3 factors. Name them.
- Vascular endothelium
- Platelets
- Coagulation system
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3 Important things to garner in history in regards to bleeding.
- Personal or family history (of bleeding)
- When (the bleeding epsiode started)
- Medications
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Quinine and quinidine can contribute to___
excessive bleeding
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In your physical exam, you want to look for signs related to?
Capillary fragility
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Petechia that do no blacnh with pressure
angiomas
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Petechiae that are not palpable
Vasculitis
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What type of bleeding defects generally have imediate onset of bleeding after trauma? (predominantly in skin, mucous membranes, nose, GI tract, urinary tract)
Platelet Defects
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What type of bleeding defects have "deep" bleeding (in joint spaces, muscles, and retroperitoneal spaces)? How are these observed on exam?
- Coagulation defects
- Observed on exam as hematomas and hemarthroses
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Name the Vit. K dependent factors.
2, 7, 9, 10
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Does prolonged bleeding time predict excess surgical blood loss?
No
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Measures rate of fibrinogen conversion to fibrin.
Thrombin Time
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Rare congenital abnormalities on synthesis or release of secretory granules.
Inherited platelet disorder
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Platelets with no alpha granules.
Gray platelet syndrome
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Inherited platelet disorders:
Thrombocytopenia
Large platelets
Neutrophils- What are these known as?
- May-Hegglin
- Neutrophils = Dohle bodies
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Inherited Platelet Disorders:
Congenital deficiency or abnormality of GPIIb-IIIa
Glanzmann's thrombasthenia
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Inherited Platelet Disorders:
Congenital deficiency or abnormality of GP Ib
Bernard-Soulier Syndrome
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Name 3 acquired platelet disorders.
- Decreased production
- Increased destruction
- Poor aggregation
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Name 2 Non-immune mediated types of platelet destruction.
- DIC
- Microangiopathic hemolytic anemia
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_____ is a diagnosis of exclusion.
ITP
-
-
Inherited Coagulation Factor Bleeding Disorders (2)
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Most common hereditary coagulation disorder, AD
vWF
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Where is von Willebrand Factor synthesized?
endothelium and megakaryocytes
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What factor does vWF carry?
8
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What anchors platelets to subendothelium and forms a bridge between platelets?
vWF
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vWD: Partial quantitative deficiency
Type I (decreased)
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vWD: Qualitative deficiency
vWF antigen is______, vWF activity is _______
- Type 2 (abnormal)
- Normal, decreased
-
vWD: Total quantitative deficiency
Type 3 (absent)
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DDAVP is not used to treat what type of vWD?
Type 2
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vWD tx:
Cryoprecipitate (source of fibrinogen, factor VIII and vWF)
Factor 8 concentrate (Humate-P or Koate-HS)
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Only plasma fraction that consistently contains vWF multimers.
Cryoprecipitate
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Prolonged bleeding after surgery or dental extractions
Hemophilia
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Hemophilia B tx:
High purty factor IX, recombinant human factor IX
-
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What is a good standard minimum of hemoglobing for men? women?
-
What images do you use to study RBCs?
Blood smear
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Disorder of globin synthesis (can't make alpha and beta chains correctly)
Thalassemia
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Can't make membrane correctly.
Hereditary spherocytosis, hereditary elliptocytosis
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Problems synthesizing heme
Porphyrias
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Impaired DNA synthesis due to a vitamin deficiency.
Megaloblastic anemia
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Make faulty, dysfunctional hemoglobins.
Hemoglobinopathies
-
Key quantitative counts of CBC?
RBC, Hb, Hct
-
Key descriptive elements of CBC?
MCV, RDW (red cell distribution width)
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Microcytic Anemias
- Iron-deficiency
- Thalassemia
- Long-standing anemia of chronic disease (mildly microcytic --> lots of time normocytic)
-
Most important test for Iron-deficiency anemia.
Ferritin
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Megaloblastic Anemias describe smears with
hypersegmented neutrophils and oval macrocytes (B12, folate deficiency)
-
Causes of Nonmegaloblastic macrocytosis
- Liver disease
- Alcohol
- MDS (Myelodysplastic Syndrome)
- Hypothyroidism
- COPD
-
Neurologic deficits can occur prior to
B12 and folate anemia (B12 levels can be normal despite a tissue deficiency)
-
Are indicated for borderline/equivocal values of B12 & folate.
MMA/homocysteine
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