Term
| At what platelet level is a pt at risk for spontaneous bleeding/bruising? |
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Definition
| This occurs when platelet levels drop below 10,000-20,000. |
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Term
| What drug is given for thrombotic complications w/essential thrombocytosis? |
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Definition
| ASA is given for thrombotic complications. |
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Term
| To what can essential thrombocytosis progress? |
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Definition
| This can progress to myelofibrosis of acute leukemia. |
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Term
| What is an autoimmune conditions in which IgG ab binds to platelets, which are removed by spleen? |
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Definition
| This is the pathogenesis of ITP. |
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Term
| In what populations is ITP common? |
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Definition
| This is commonly seen in children after viral illnesses. In adults it occurs in a chronic form not related to viral infections. |
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Term
| What is the worst complication of itp? |
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Definition
| The worst complication is spontaneous ich. |
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Term
| 12 yo m recovering from flu presents w/mucosal bleeding. CBC shows hct 50, hgb 14, nml rbc, nml wbc, platelets 12,000, mcv nml, mchc nml, mch nml, nml rdw, nml retics. Bone marrow is nml, coags are nml. Megathrombocytes are present on smearl. What do you suspect, how do you tx, and what is the worst complication? |
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Definition
| This clinical scenario describes ITP. Worst complication is spontaneous ich. Tx w/high dose prednisone. If platelets less than 10,000 give platelets for short term. |
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Term
| What disorders comprise thrombotic microangiopathy? |
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Definition
| This is comprised of ttp and hus. |
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Term
| What coagulopathy involves a deficiency in ADAMTS13 (vWf- cleaving protease)? In what does this result? |
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Definition
| This causes TTP by causing the accumulation of vWf mulitmers w/platelet agglutination and aggregation. |
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Term
| What are 2 triggers of TTP? |
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Definition
| 2 triggers are mes (quinine, ticlopdipine) or infection (hiv). |
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Term
| What are the clinical findings of TTP and HUS? |
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Definition
| The clinical findings are CNS changes (only one), Renal failure, Anemia, Fever, and Thrombocytopenia. |
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Term
| What are the 2 characteristics of HUS and TTP? |
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Definition
| The 2 characteristics are incorporation of thrombi into clots in microvasculature and microangiopathic hemolytic anemia. |
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Term
| What disease is associated w/diarrheal illness in ids (shigella, salmonealla, and *ecoli 0157h7*)? |
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Definition
| This is associated w/HUS. |
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Term
| What symptom differs between HUS and TTP? |
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Definition
| HUS does not involve CNS changes. |
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Term
| What disease occurs w/postpartum state or secondary to meds in adults (estrogen, cyclosporine or tacrolimus)? |
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Definition
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Term
| How do coags and coombs test results change w/TTP and HUS? |
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Definition
| These lab values are nml w/TTP and HUS. |
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Term
| Is thrombocytopenia more severe in HUS or TTP? |
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Definition
| This is more severe in TTP. |
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Term
| Is thrombocytopenia more severe in HUS or TTP? |
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Definition
| This is more severe in TTP. |
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Term
| Is renal failure more severe in HUS or TTP? |
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Definition
| This is more severe and longstanding in HUS. |
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Term
| F pt c/o tiny purplish spots all over the body after having a "stomach flu." She has a fever of 101.5, pulse 110, bp 100/70, and respirations 20. Labs show hct 30, hgb 10, low rbc, nml wbc, platelet level of 85,000, nml mchc, nml mcv, nml mch, increased reticulocytes, increased rdw w/schistocytes in blood smear, elevated ld, elevated indirect bilirubin, decreased haptoglobin, negative direct antibody test, elevated creatinine, nml coags, and nml coomb's test. What do ou suspect, and how do you tx? |
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Definition
| This clinical scenario describes thrombotic microangiopathy. Tx: ACT FAST. Tx w/plasmapheresis. Mortality rate is >95% w/o tx. |
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Term
| What is the MOST COMMON drug induced thrombocytopenia? |
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Definition
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Term
| In what disease does IgG Ab react w/PF4 in a complex w/this drug? |
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Definition
| This reacts w/Heparin (and less commonly w/lovenox) in HIT. |
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Term
| What causes prothrombotic effects in HIT? |
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Definition
| This is caused by platelet microparticle release. |
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Term
| What can be life threatening in HIT? |
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Definition
| Arterial or venous thrombotic events can be life threatening in this dz. |
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Term
| Pt presents w/new oneset of thrombosis 5 days s/p admission for dvt. Hgb 48, hct 16, nml rbc, nml wbc, platelets 75,000, mch nml mchc nml, mcv nml, retics nml, rdw nml. How do you dx and how do you tx? |
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Definition
| This clinical scenario indicates HITT, a potentially life threatening form of HIT. Dx: Abs to PF4/Positive HIT panel. Tx: DISCONTINUE ALL HEPARIN PRODUCTS. Use alternative anticoagulants (like leprudin or argatroban). |
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Term
| what is caused by unregulated clotting and anticlotting cascades? |
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Definition
| This is the pathology behind DIC. |
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Term
| What are 4 causes of DIC? Which is most common? |
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Definition
| This can be caused by *sepsis: most common*, burns, obstetric complications, or CA (acute promyelocytic leukemia (M3). |
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Term
| Which is morre common in DIC: thrombotic or bleeding complications? |
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Definition
| Bleeding complications more common than thrombotic complications. |
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Term
| What is Trousseau's syndrome? |
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Definition
| This occurs w/subacute DIC in pts w/pancreatic CA. |
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Term
| Pt being tx for septic shock begins bleeding from every IV and wound site. Pt has decreased fibrinogen, increased FDP and D-Dimer, thrombocyte level of 15,000, and increased PT and aPTT times. Blood smear shows shcitocytes. What do you suspect and how do you tx? |
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Definition
| This clinical scenario indicates DIC. Tx underlying dz! Use heparin if thrombosis is present and platelets are over 50,000. Use platelet transfusion to bring counts to greater than 50,000 if bleeding present. Correct coagulopathy w/FFP if bleeding present. |
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Term
| What is the genetic cause of Hemophilia? |
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Definition
| X-linked recessive pattern of inheritance affects only males typically and results in deficient or defective factor VIII or IX. |
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Term
| What is the classification if Factor VIII levels are less than 1%? |
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Definition
| This is classified as severe Hemophilia A. |
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Term
| What is the classification if Factor VIII levels are 1-5%? |
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Definition
| This is classified as moderate hemophilia A. |
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Term
| What is the classification if Factor VIII levels are greater than 5%? |
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Definition
| This is classified as mild hemophilia A. |
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Term
| What is the most severe bleeding disorder? |
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Definition
| The most severe is Hemophilia A. |
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Term
| Where does bleeding occur w/hemophilia A? |
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Definition
| Bleeding occurs into jts, mm, and gi tract. |
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Term
| What dz often accompanies hemophilia? |
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Definition
| Many are now HIV + d/t past contamination of factor VIII products. HIV associated w/thrombocytopenia may make bleeding worse. |
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Term
| 1 yo boy presents w/severe bruising in right elbow after bumping it on a table. PTT is prolonged, bleeding time is prolonged, vWF and fibrinogen levels are nml. How do you dx and how do you tx? |
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Definition
| This clinical scenario could indicate hemophilia. Ask about family hx and dx w/ low Factor VIII or Factor IX activity level in the absence of other conditions. Tx w/recombinant factor VIII infusions or Factor IX concentrates. |
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Term
| Which is more common: hemophilia a or b? |
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Definition
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Term
| What can be used form mild hemophilacs going for minor sx? |
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Definition
| These pts can use DAVP, vWf to protect factor VIII. |
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Term
| How can refractory hemophiliacs be tx? |
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Definition
| Refractory pts can be tx w/EACA. |
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Term
| What additional condition w/hemophilia makes the dz more difficult to tx and causes the prolonged PTT to remain uncorrected w/addition of nml plasma? |
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Definition
| This occurs w/factor VIII inhibitor. |
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Term
| How do you tx acquired factor VIII Abs? |
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Definition
Hemophiliacs: tx w/immune tolerance induction. If life threatening, use plasmapheresis and high dose factor VIII infusions. Can use Factor VII concenrates to flood the system.
Spontaneous: tx w/cyclophosphamide, prednisone, Factor VIII and or Factor VII concentrates. |
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Term
| The liver is the site of all clotting factors except which one? How do you tx coagulopathy of liver dz? |
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Definition
| This is the site of synthesis of all clotting factors except VIII. Tx w/ffp or platelet transfusions as appropriate. |
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Term
| How does vitamin K deficiency occur? |
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Definition
| This occurs in pts w/dietary deficiency or on abx. |
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Term
| In vitamin K deficiency, which is more prolonged: PT or PTT? |
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Definition
| PT more prolonged than PTT. |
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Term
| How do you distinguish vit K deficieny from warafin tx? |
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Definition
| Distinguish by response to vitamin k therapy. |
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Term
| What is an AR disorer w/lack of Gp IIb/IIIa receptors w/defective platlet aggregation that causes mucosal and postop bleeding, prolonged blleding time? How do you tx? |
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Definition
| This is Glanzman's thrombasthenia; tx w/platelet transfusion. |
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Term
| What is an AR disorder w/lack of GpIb receptor, which mediates platelet adhesion and causes prolonged bleeding time, severe mucosal and postop bleeding? How do you tx? |
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Definition
| This is bernard-soulier dz; tx w/platelets. |
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Term
| What is defective secretion of platelet granules needed for platelet aggregation that causes increased post op bleeding, prolonged bleeding time, and is worsened by ASA? How do you tx? |
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Definition
| This is storage pool dz; tx w/platelet transfusion to correct bleeding. |
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Term
| What causes abnml platelet function, mucosal and gi bleeing and is improved by dialysis? What else is helpful? |
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Definition
| Uremia is helped by DDAVP, which can release vWf. |
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Term
| Pt comes from an Ashkenazi Jewish family w/ family hx of bleeding and presents w/epistaxis that will not stop. What do you suspect and how do you tx? |
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Definition
| This clinical scenario indicates Factor XI deficiency. #1 tx is FFP. |
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Term
| How is von Willebrand's dz aquired? What is important when taking hx? |
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Definition
| This has an autosomal pattern of inheritance. Obtain a family hx (any family members w/bleeding postop?) |
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Term
| What causes von Willebrands dz? |
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Definition
| This is caused by deficient or defective vWf, which is needed or platelet adhesion and is synthesized in megakaryocytes and endothelial cells. |
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Term
| Where is vWf syntehsized and what does it protect? |
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Definition
| This is synthesized in megakaryocytes and endothelial cells and protects factor VIII. |
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Term
| Pt w/family hx of postop bleeding presents w/mucosal bleeding. Pt has prolonged bleeding time, nml Pt and aPTT. What do you suspect and how do you tx? |
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Definition
| This clinical scenario indicates von Willebrand dz, and demonstrated by low vWf. Tx w/DDAVP and Factor VIII concentrates. In dental procedures use EACA (he's never used this). |
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