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81 Cards in this Set
- Front
- Back
What are two types of Hemorrhagic disorders you can have?
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-Primary
-Secondary |
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What are Primary hemorrhagic disorders?
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Disorders of platelet PLUG formation - the INITIAL thing that forms in clotting
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What is the characteristic sign of a defect with initial plt plug formation?
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Petechial hemorrhages - pinpoint or punctate hemorrhages on SKIN and MUCOUS MEMBRANES
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What are multiple petechial subcutaneous hemorrhages sometimes described as?
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A RASH
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What lab tests are indicative of defects of primary hemostasis - platelet plug formation?
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Prolonged BLEEDING times - but there won't be a prolonged PT or PTT
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What are 2 classes of disorders of primary hemostasis?
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-Vascular lesions
-Platelet disorders |
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Which of the two is more likely to have an abnormal bleeding time?
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Platelet disorders - vascular lesions rarely have abnormal lab findings
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What is Simple purpura? Where is it found, on whom?
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Easy bruising - esp on the upper thighs, in otherwise healthy persons
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What is it called when there are hemorrhagic areas on the backs of hands and forearms of older people?
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Senile purpura
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Why do older people tend to get senile purpura?
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Because of age-dependent atrophy of the vascular supportive tissues
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What vitamin deficiency leads to vascular lesions?
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Vit C deficiency - scurvy
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Where are hemorrhages typically seen in scurvy?
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-Gingival
-Muscles -Subcutaneous tissues |
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What does Scurvy do to the little hairs on the body? Why?
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Makes them twisted and corkscrewy - because of hemorrhagic Perifollicular hyperkeratotic papules that develop
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What is a form of hypersensitivity vasculitis that is seen in young children?
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Henoch-Schonlein purpura
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What is synonymous with hypersensitivity vasculitis?
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Leukocytoclastic angiitis
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And what causes the purpura in Henoch-Schonlein?
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An immune reaction that damages the vascular endothelium
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What are 4 clinical findings in Henoch Schonlein?
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1. Palpable purpura - Hemorrhagic urticaria
2. Fever 3. Arthralgias 4. GI/Renal involvement |
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What patient population is associated with Hereditary hemorrhagic telangiectasia?
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Osler-Weber-Rendu - in Mormons of Utah
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What is the inheritance of Osler-Weber-Rendu?
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Autosomal dominant
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What is clinically seen in Osler Weber Rendu?
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Localized malformations of venules and capillaries of the skin and mucous membranes leading to hemorrhages
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What connective tissue disorder can lead to vascular bleeding?
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Ehlors-Danlos
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What plasma dyscrasia can lead to vascular damage?
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Waldenstrom macroglobulinemia
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How does Waldenstrom's macroglobulinemia lead to vascular damage?
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Due to the hyperviscosity of blood, and also platelet abnormalities
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What other systemic condition can cause vascular damage?
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Amyloidosis
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What are 2 bacterial infections that lead to primary hemorrhagic disorders?
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-Rickettsial diseases - RMSF
-Meningococcal diseases |
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Why do Rocky mtn spotted fever and Meningococcemia cause primary hemostasis disturbance?
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Because they involve the vascular endothelium and lead to necrosis and rupture of small blood vessels
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What are 2 types of PLATELET disorders that can lead to Primary Hemostasis dysfunction?
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1. Thrombocytopenia
2. Platelet FUNCTION abnormality |
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What are 3 characteristic findings in Thrombocytopenia?
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-Cutaneous petechiae
-Intracranial bleeds -Mucosal oozing |
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What lab findings are seen in Thrombocytopenia?
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-Decreased Plt count
-Increased Bleeding Time |
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What will cause differences in Megakaryocyte counts in the BM in thrombocytopenia?
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Whether it is due to decreased production, or increased destruction.
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What are 4 conditions that show decreased Platelet production?
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-Radiation
-Acute leukemia - replaces BM -Myelopthesis -Aplastic anemia |
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What are 3 conditions that affect circulating platelets, rather than production?
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-Multiple transfusions - dilutes
-DIC - uses them all up -Splenic sequestration |
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What is TTP?
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Thrombotic thrombocytopenic purpura
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What happens in TTP?
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Platelets form hyaline microaggregates along the vessels causing narrowing!
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What happens as a result of the narrowed vessels?
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RBCs get hemolysed - microangiopathic HA
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So what are findings on a PBS in TTP?
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-Helmet cells
-Schistocytes -Low platelets |
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What are 3 other developments often in TTP?
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-Transient neurologic abnormlts
-Renal insufficiency -Fever |
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What is a CAUSE of TTP?
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Deficiency in ADAMTS 13 - vWF metalloproteinase
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What is the result of LACKING the metalloproteinase of vWF?
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Large very high-MW multimers of vWF which PROMOTES platelet aggregation
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What is ITP?
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Idiopathic or Immune Thrombocytopenic Purpura
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How is ITP different when in Children vs Adults?
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Children - follows a viral infection; acute, self limiting
Adults - Chronic |
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What is ITP CAUSED by?
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Auto-antibodies that coat plts and then cause splenic removal
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What can cause FETAL ITP?
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Maternal antibody crossing the placenta
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What are the 2 diagnostic features of ITP?
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-Decreased platelets
-Increased Megakaryocytes |
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What are the 2 hallmark findings in Platelet FUNCTION abnormalities?
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-Normal platelet count
-Increased BT |
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What are 2 things that cause Abnormal Platelet FUNCTION?
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-Abnormal plt ADHESION
-Abnormal plt AGGREGATION |
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What is the disease of abnormal platelet adhesion?
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Bernard soulier - lack of GP1b/a receptor for vWF so platelets can't adhere to exposed collagen
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What is seen on the PBS in Bernard soulier?
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Large platelets
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What is an inherited disorder of platelet aggregation?
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Glanzmann's thrombasthenia - lack of GP2b/3a so fibrinogen can't crosslink platelets with fibrinogen bridges
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What causes ACQUIRED disorder of platelet aggregation?
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Aspirin - inhibits COX causing lack of TxA2
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What causes 2ndary hemostasis disorders?
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Deficiency of CLOTTING factors
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How are symptoms of 2ndary hemostasis disorders different from those of Primary?
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Involve bleeding from LARGER vessels - not pinpoint/petechiae
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What are 4 common manifestations of 2ndry Hemostatic disorders?
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-Hemarthroses
-Large hematomas -Large ecchymoses -Extensive bleeding in traumas |
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How are the LAB findings different in 2ndry vs primary hemostatic disorders?
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-BT will be NORMAL
-Platelets are usually normal -PT and/or PTT will be prolonged |
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What will a prolonged PT indicate?
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Deficiency of Fibrinogen, or Factors 2, 5, 7, or 10
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What will a prolonged PTT indicate?
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Deficiency of ALL factors EXCEPT 7 or 13
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What does a prolonged Thrombin Time indicate?
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Deficiency of Fibrinogen
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What is Classic Hemophilia?
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Fx 8 deficiency - Hemophilia A
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What is the inheritance pattern of Classic hemophilia?
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X-linked
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What is diagnostic of Hemophilia A?
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Prolonged PTT that corrects with normal plasma
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What is indistinguishable from Hemophilia A?
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Christmas disease - Fx 9 defic, Hemophilia B
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What is the most COMMON cause of a prolonged PT in adults?
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Lack of Vit K
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What causes the lack of Vit K?
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FAT Malabsorption due to:
-Pancreatic disease -Small bowel disease |
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What causes lack of Vit K in NEONATES?
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Lack of gut flora to make it!
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What does Vit K deficiency in neonates lead TO?
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Hemorrhagic disease of the Newborn
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What does Hemorrhagic dis of the Newborn result in?
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Lack of all the Vit K dep clotting factors, so prolonged PT AND PTT!
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What is the most COMMON hereditary bleeding disorder?
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von Willebrand disease
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Where is vWF made?
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-Endothelial cells
-Megakaryocytes |
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What is vWF FOR?
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-Carries Fx 8
-Platelet adhesion |
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So what type of a disorder is LACK of vWF?
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Mixed - both primary and secondary hemostatic disorder!
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What are the characteristic findings in vWD?
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-Abnormal BT - lack adhesion
-Prolonged PTT - lack Fx VIII |
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What is DIC?
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Widespread clotting leading to consumption of Platelets, Fx's 2, 5, 8, and Fibrinogen!
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What are the clinical manifestations of DIC?
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Thrombosis PLUS hemorrhage
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What lab tests will be abnormal in DIC?
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ALL OF THEM
-BT prolonged -PT/PTT -Thrombin time Tct -FSP's and D-dimer |
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When is DIC most COMMONLY seen?
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Obstretic complications!
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What is a systemic condition that leads to coagulopathy?
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LIVER DISEASE
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Why does liver disease lead to coagulopathy?
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Because ALL clotting factors are made there except vWF!
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What lab findings will develop as hepatocellular disease progresses?
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PT/PTT/TT will all prolong
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What can be used to help alleviate the clotting factor deficiencies in liver disease?
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Vit K derivatives
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How do Multiple transfusions cause coagulopathy?
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Dilutional - if they lack platelets and factors
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What is the clinical manifestation of Dilutional coagulopathy?
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Persistent bleeding from surgical wounds
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