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29 Cards in this Set
- Front
- Back
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What does bleeding stoppage require?
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-blood vessel contraction, platelet adhesion, plug formation and plasma
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What is typically seen w/ hemorrhagic disease due to increased vascular fragility?
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-petechiae and purpura in skin, GI, GU
-hemorrhage into jts, muscles and sub Q tissues, usually not serious -bleeding time, platelet count, and coagulation time are all normal |
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Osler -Weber-Rendu Syndrome
aka Hereditary Hemorrhagic Telangiectasia |
-microvascular dilations in the skin, mucosa, and GI tract
-petechiae are often noted on the lips and tongue -CONGENITAL |
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Ehlers-Danhlos disease
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-mesenchymal defect in collagen synthesis
-leads to less blood vessel support -CONGENITAL |
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Henoch - Scholein Purpura
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-allergic purpura (purpura are larger in size than petchiae but this disease can also manifest w/ petechiae in the oral area
-a/w jt and GI probs, generalized vasculitis, immune complexes deposit in BVs and in glomerular mesengial regions (renal failure) |
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Acquired types of hemorrhagic disease due to increased vascular fragility:
Infections and drugs |
-capillary damage after meningococcal, bacterial endocarditis or measles lead to joint and GI probs
-immune complex deposition can also lead to drug induced vascular damage (especially antihistamines) |
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Acquired types of hemorrhagic disease due to increased vascular fragility:
Miscellaneous |
-cushing's syndrome
-protein starvation -scurvy -senile purpura |
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Thrombocytopenia
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-when less than 150,000 platelets/dl
-when >20,000/dl spontaneous bleeding occurs |
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Decreased platelet production
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-decreased megakaryocytes in BM (ex. aplastic anemia, drugs, or malignancy)
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What are 4 examples of decreased platelet survival?
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-platelets don't survive more than 4 hours
1. immune reaction to antimalarials 2. Infxns (mono or HIV) 3. Autoimmune ITP or SLE) 4. Thrombotic microangiopathies/consumption (DIC, TTP, MAHA) |
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Idiopathic Thrombocytopenia Purpura
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-IgG antiplatelet Abs on surface of platelets, destroyed in the spleen
-*Platelet # decreases leading to an increased bleeding time*** -in kids after viral infxn, can be chronic in young women -Dx: inc megakaryocytes in BM |
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****Thrombotic Microangiopathies:
Hemolytic Uremic Syndrome |
-fibrin-platelet microthrombi deposit in arterioles and capillaries--> fever, thrombocytopenia, MAHA, renal fail
-***childhood, NO neruo deficits, acute renal failure follows GI infx |
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****Thrombotic Microangiopathies:
Thrombotic Thrombocytopenic Purpura |
-fibrin-platelet microthrombi deposit in arterioles and capillaries--> fever, thrombocytopenia, MAHA, renal fail
-***commonly seen in females, NEURO deficit, no evidence of previous bacterial infx |
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Isoimmune Thrombocytopenia:
Neonatal thrombocytopenia |
-in newborn resulting from development of IgG Abs against PL A1 Ags in fetus
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Isoimmune Thrombocytopenia:
Post Transfusion thrombocytopenia |
-platelets are destroyed when blood w/ PL A1- positive platelets transfused to pts who are PL A1 - negative, but has been sensitized to PL A1 Ag by previous transfusion or pregnancy
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Hemorrhagic diseases due to defective platelets:
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Inherited:
1. Bernard-Soulier Syndrome 2. Glanzman disease Acquired 1. drugs (nsaids)--> defective release of ADP by platelets |
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***Bernard - Soulier Syndrome
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-***defective platelet adhesion
-deficient in glycoprotein Ib (receptor for vWF) leads to increased bleeding time and decreased platelet number (normal PT and PTT) |
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***Glanzman disease
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-***impaired platelet aggregation; platelets may adhere but no plug formed
-autosomal recessive and platelets lack glycoprotein IIb/IIIa with increased BT (normal PT and PTT) |
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***Essential Thrombocytopenia
aka Idiopathic Thrombocytopenia |
***least common MPD
-megakaryocyte hyperplasia in BM -increased platelet count w/ giant platelets and abnormally formed platelets (fxn of platelets is impaired) |
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Overview of Hemorrhagic Diseases due to Abnormalities in Clotting factors
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-deficiency in coag factors leads to ecchymosis, hematomas in subQ tissue, prolonged bleeding after surgery, and hemorrhage into jts, GI and GU
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Prothrombin time (PT) vs Partial thromboplastin time (PTT)
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-PT measures coagulation factors in the extrinsic pathways (i, II, V, VII, X) (Factor VII is unique to extrinsic)
-PTT measures coagulation factors of the intrinsic pathway (8, 9, 11, 12 unique to the intrinsic) |
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***Hemophilia A
(Factor VIII deficiency, Classical Hemophilia) |
-X linked recessive (majority), 30% due to new mutation
-bleeding: trauma or spontaneously- joints, muscles internal organs, brain -severe: factor VIII< 1% of normal |
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***Diagnosis of Hemophilia A
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-normal BT (normal platelets), normal PT (NO extrinsic factors affected), normal platelet count
-PTT IS PROLONGED (correct w/ mixing normal plasma bc now have factor VIII) -Factor VIII assay for Dx |
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***Hemophilia B
(Factor IX deficiency, Christmas disease) |
-X-linked recessive
-indistinguishable from hemophilia A but less common -asymptomatic or hemorrhages -***Prolong PTT w/ normal bleeding time -DX only by Factor IX assay |
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Von Willebrands disease
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-auto dominant
-epistaxis, menorrhagia, abnL bleeding after surgery -NO BLEEDING in JT SPACES |
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***Dx of Von Willebrands disease
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-BT is prolonged and platelet count is normal (bc platelets can't adhere to injury due to dec vWF)
-PTT prolonged due to decrease in factor VIII |
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Type 1 vs Type 2 Von Willebrands disease
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-Type 1 (most common) has decreased quantity of vWF, which normally stabilizes factor VIII, (Fact VII also decreased)
-Type 2 less common (qualitative defect in vWF |
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Acquired defects of Coagulation
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1. Vit K def--> dec factors II, VII, IX, X
-severe liver disease and anticoag therapy can cause defects and severe hemorrhage |
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ITP vs TTP
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1. ITP: autoimmune, young females, kids, IgG Abs on platelets
2. TTP: unknown, only young females, Fibrin, platelet microthrombi |
