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32 Cards in this Set
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Disorders of 1° homeostasis (char)
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Defect platelet plug formation: vascular lesions, thrombocytopenia, platelet dysfunction. Prolonged bleeding time (NL PT, PTT). Sx: Petechia, muscosal oozing (epistaxis, gums, GI)
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Simple purpura
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Vascular cause of 1° homeostatis disorder Easy bruising
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Senile purpura
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Vascular cause of 1° homeostatis disorder. Age-dependent atrophy of vascular supportive tissues
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Scurvy
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Vascular cause of 1° homeostatis disorder. Ascorbic acid def => gingival hemorrhage, eccyhmosis, hemorrhagic perifollicular hyperkeratotic papules (each surrounding corkscrew-like hair). Stores last 1-3 months.
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Henoch-Schonlein purpura
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Vascular cause of 1° homeostatis disorder. "Allergic purpura". Hypersensitivity vasculitis (leukocytoclastic angiitis) => hemorrhagic urticaria (palpable purpura), fever, arthralgias, GI, renal. IgA.
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Osler-Weber-Rendu
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Hereditary hemorrhagic telangiectasia. Vascular cause of 1° homeostatis disorder. Localized malformations of venules + capilaries.
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Ehlers-Danlos syn
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Collagen or elastin defect. Vascular bleeding, articular hypermobility, dermal hyperelasticity, tissue fragility (berry aneurysms).
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Waldenstrom macroglobulinemia
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Vascular cause of 1° homeostatis disorder. Sluding of hyperviscous blood => vascular damage. Also defects in platelet func.
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Amyloidosis (vascular effects)
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Vascular cause of 1° homeostatis disorder. vessel damage.
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Rickettsia (vasc)
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Vascular cause of 1° homeostatis disorder. Rock Mountain spotted fever: necrosis of small vesslels
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Meningococcus (vasc)
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Vascular cause of 1° homeostatis disorder. necrosis of small vessels.
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Thrombocytopenia
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Sx: petechia, intracranial bleeding, mucosal oozing. Prolonged bleeding time (NL PT, PTT). Marrow: if inc destruction => inc megakaryocytes; dec production => dec megakaryocytes. Causes: radiation, drugs, acute leukemia, myelophthisis, aplastic anemia (benzene, CD8+ autoimmune), splenic sequestration, dilution (transfusion), DIC, ITP, TTP
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Idiopathetic thrombocytopenic purpura
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Immune thrombocytopenic purpura. Anti-platelet ab => spelnic mphage. Maternal IgG => fetal thrombocytopenia. Dx: inc megakaryocytes, no palpable splenomegaly. Kids: acute, self-limiting post-infection. Adults: chronic.
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Thrombotic thrombocytopenia purpura
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ADAMTS 13 (vWF metalloprotease) def => platelet-derived hyaline microaggregates in small vessels => 1) fever 2) thrombocytopenia, 3) microangiopathic hemolytic anemia, 4) renal insufficiency, 5) neuro abn. Helmet cells, schistocytes.
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ADAMTS 13
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vWF metalloprotease. Defeciency = TTP => HMW vWF acculumates => platelet microaggregates
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Bernard-Soulier
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Platelet dysfunction. AR. GPIb-IX-V def (platelet surface glycoprotein req for platelet adhesion).
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Glanzmann thrombasthenia
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GPIIb-IIIa def (platelet surface glycoprotein req for fibrinogen bridges)
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Disorders of 2° hemostasis (char)
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Plasma cloting factor deficiency. Large vessel bleeds: hematomas, large eccyhmoses, hemarthroses, delayed bleeding.
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Classic hemophilia
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Hemophilia A. Factor 8 def. XR. Variable severity. Inc PTT corrected w/ NL plasma. (NL PT, TT). Tx: recombinant 8, DDAVP
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Christmas disease
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Hemophilia B. Factor 9 def. XR Less common than hemophilia A, but same clinical feat.
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Vitamin K def
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Often Fat malabsoprtion (panc, SI). Neonates: hemorrhagic dz of the newborn (def exogenous K + incomplete intestinal colonization by K-synth bact). Dec 2, 7, 9, 10. Inc PT, PTT.
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von Willebrand dz
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Most common hereditary bleeding disorder. 1° + 2° defect. AD or AR (many var). Synth by endothelium + megakaryocytes, carries factor 8, mediates platelet adhesion to sebendo (injury) binding GPiB-IX-V. 1) platelet adhesion fails => inc bleeding time. 2) functional factor 8 def => inc PTT.
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Disseminated intravascular coagulation
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Release thromplastin (TF)/* intrsinsic => Consume platlets + coag factors (2, 5, 7) + fibrinogen => thrombosis + hemorrhage + microangiopathic hemolytic anemia (schisto). Inc fibrin/fibringoen degradation (split) products, inc bleeding time, PT, PTT, TT. Causes: obstetric complications (amniotic fluid emboli, retained dead fetus, abruptio placentae) , cancer (trosseau syndrome), infection (GN sepsis), trauma, immune (IC, hemolytic transfusion rxn).
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Liver disease
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Dec all factors !vWF. Inc Pt, PTT, thrombin, bleeding time (platelet functional defect or thrombocytopenia). Tx: Vit K derivatives => carboxylation of glutamate of 2, 7, 9, 10.
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Dilutional coagulopathy
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Multiple transfusions (defiicent in platelets, 2, 5, 8) => persistent bleeding from surgical wounds. Thrombocytopenia or inc PT or inc PTT
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TAR syndrome
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congenital thrombocytopenia with bilateral absent radius. Possibly AR.
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Factor V Leiden
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Most common hereditary thrombophilia. Mutated APC-binding site on factor V. "Hereditary resistance to activated protein C"
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Prothrombin 20210A transition
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2nd most common herditary thrombophilia. G=>A mut 3'UTR prothrombin => elevated prothrombin levels
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Methylene tetrahydrofolate reductase mut
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MTHFR C677T. Hereditary thrombophilia. Inc homocysteine => arterial + venous thrombosis. A/w nerual tube defects, neoplasms. Tx: folate, B6 (pyridoxine), B12 (cobalamin).
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Elevated factors causing thrombophilia
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VIII, IX, XI
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Antiphospholipid antibody sydrome
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Prothrombotic disorder. Polyclonal ab vs proteins bound to PLs. 1) Lupus anticogalant (vs prothrombin) 2) anticardiolipin (vs B2GPI) causes FP VDRL. Both a/w Lupus. Dx: Inc PTT (paradoxical). Sx: recurrent venous + art thromboembolism, fetal loss, thrombocytopenia, neuro.
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Heparin-induced thrombocytopenia
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Thrombosis. Ab vs heparin-PF4 complex => bind FcRgamma on platelets => * platelet
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