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32 Cards in this Set

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  • Back
Disorders of 1° homeostasis (char)
Defect platelet plug formation: vascular lesions, thrombocytopenia, platelet dysfunction. Prolonged bleeding time (NL PT, PTT). Sx: Petechia, muscosal oozing (epistaxis, gums, GI)
Simple purpura
Vascular cause of 1° homeostatis disorder Easy bruising
Senile purpura
Vascular cause of 1° homeostatis disorder. Age-dependent atrophy of vascular supportive tissues
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.
Henoch-Schonlein purpura
Vascular cause of 1° homeostatis disorder. "Allergic purpura". Hypersensitivity vasculitis (leukocytoclastic angiitis) => hemorrhagic urticaria (palpable purpura), fever, arthralgias, GI, renal. IgA.
Hereditary hemorrhagic telangiectasia. Vascular cause of 1° homeostatis disorder. Localized malformations of venules + capilaries.
Ehlers-Danlos syn
Collagen or elastin defect. Vascular bleeding, articular hypermobility, dermal hyperelasticity, tissue fragility (berry aneurysms).
Waldenstrom macroglobulinemia
Vascular cause of 1° homeostatis disorder. Sluding of hyperviscous blood => vascular damage. Also defects in platelet func.
Amyloidosis (vascular effects)
Vascular cause of 1° homeostatis disorder. vessel damage.
Rickettsia (vasc)
Vascular cause of 1° homeostatis disorder. Rock Mountain spotted fever: necrosis of small vesslels
Meningococcus (vasc)
Vascular cause of 1° homeostatis disorder. necrosis of small vessels.
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
Idiopathetic thrombocytopenic purpura
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.
Thrombotic thrombocytopenia purpura
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.
vWF metalloprotease. Defeciency = TTP => HMW vWF acculumates => platelet microaggregates
Platelet dysfunction. AR. GPIb-IX-V def (platelet surface glycoprotein req for platelet adhesion).
Glanzmann thrombasthenia
GPIIb-IIIa def (platelet surface glycoprotein req for fibrinogen bridges)
Disorders of 2° hemostasis (char)
Plasma cloting factor deficiency. Large vessel bleeds: hematomas, large eccyhmoses, hemarthroses, delayed bleeding.
Classic hemophilia
Hemophilia A. Factor 8 def. XR. Variable severity. Inc PTT corrected w/ NL plasma. (NL PT, TT). Tx: recombinant 8, DDAVP
Christmas disease
Hemophilia B. Factor 9 def. XR Less common than hemophilia A, but same clinical feat.
Vitamin K def
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.
von Willebrand dz
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.
Disseminated intravascular coagulation
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).
Liver disease
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.
Dilutional coagulopathy
Multiple transfusions (defiicent in platelets, 2, 5, 8) => persistent bleeding from surgical wounds. Thrombocytopenia or inc PT or inc PTT
TAR syndrome
congenital thrombocytopenia with bilateral absent radius. Possibly AR.
Factor V Leiden
Most common hereditary thrombophilia. Mutated APC-binding site on factor V. "Hereditary resistance to activated protein C"
Prothrombin 20210A transition
2nd most common herditary thrombophilia. G=>A mut 3'UTR prothrombin => elevated prothrombin levels
Methylene tetrahydrofolate reductase mut
MTHFR C677T. Hereditary thrombophilia. Inc homocysteine => arterial + venous thrombosis. A/w nerual tube defects, neoplasms. Tx: folate, B6 (pyridoxine), B12 (cobalamin).
Elevated factors causing thrombophilia
Antiphospholipid antibody sydrome
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.
Heparin-induced thrombocytopenia
Thrombosis. Ab vs heparin-PF4 complex => bind FcRgamma on platelets => * platelet