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88 Cards in this Set
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- Back
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What are considered the life-threatening bleeds?
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bleeding in oropharynx
intracerebral hemorrhage |
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What is the best screening test for coagulation?
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partial thromboplastin time (PTT)
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What does PTT measure?
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intrinsic pathway
factors VIII, IX, XI, XII Heparin circulating anticoagulants |
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What does PT measure?
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extrinsic pathway
factor VII, vitamin K Warfarin |
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What test measures fibrin degradation products?
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D-dimer
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What does a negative D-dimer mean?
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no clot
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What is thrombocytosis?
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marked proliferation of megakaryocytes in bone marrow
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What are the clinical manifestations of thrombocytosis?
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Thrombosis
Erythromelalgia Bleeding, typically mucosal Splenomegaly |
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In thrombocytosis, where do the venous thrombosi usually occur?
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mesenteric, hepatic, or portal veins
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What is erythromelalgia?
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painful burning of the hands with erythema
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What lab findings would you expect to see in thrombocytosis?
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increased platelets
increased WBC, mild large platelets on peripheral smear normal RBC morphology prolonged bleeding time bone marrow - increased megakaryocytes |
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What differentiates thrombocytosis from CML?
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no Philadelphia chromosome in thrombocytosis
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What is the differential for thrombocytosis?
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secondary causes of thrombocytosis (inflammatory disorders, chronic infections, iron deficiency)
polycythemia vera myelofibrosis CML |
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How do you treat thrombocytosis?
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Hydroxyurea
Anagrelide |
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What limits the dosing of hydroxyurea?
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dose limiting neutropenia
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What is the major source of morbidity in thrombocytosis patients?
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Thrombosis
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What are the different etiologies for thrombocytopenia?
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decreased bone marrow
splenic sequestration acccelerated destruction drug induced |
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A patient presents with thrombocytopenia and a bone marrow biopsy shows decreased megakaryocytes. What might you expect as a cause of the thrombocytopenia?
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Decreased bone marrow production:
- marrow aplasia - fibrosis - infiltration with malignant cells - cytotoxic drugs |
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What percentage of the mass of platelets stay in the spleen?
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1/3 platelet mass
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What does a splenectomy causes? Splenomegaly?
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splenectomy: thrombocytosis
splenomegaly: thrombocytopenia |
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What conditions can cause accelerated destruction of thrombocytes?
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vasculitis
hemolytic uremic syndrome (HUS) ITP TTP DIC Viral or bacterial infections |
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What drugs can induce thrombocytopenia?
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chemotherapeutic agents
alcohol sulfonamides thiazides phenytoin unfractionate heparin |
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Unfractionated heparin can cause what syndrome?
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White clot syndrome: intravascular platelet aggregation and paradoxical thrombosis
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What are the clinical manifestations of thrombocytopenia?
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purpura
petechiae ecchymoses hematomas menorrhagia GI hemorrhage |
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Can thrombocytopenia be controlled by local measures?
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Yes
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What labs would you expect to see with thrombocytopenia?
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Prolonged bleeding time
Decreased platelet count |
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A patient has a platelet count less than 20,000. What would you expect to see?
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spontaneous bleeding, petechiae
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A platelet count less than 50,000 can lead to what?
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easy bruising, purpura after minor trauma, bleeding after mucous membrane surgery
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At what platelet count does bleeding time prolong?
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at less than 100,000
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What is idiopathic thrombocytopenia purpura?
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an autoimmune disorder in which an IgG autoantibody is formed that binds to platelets
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Acute ITP is most common in what age group? What causes it?
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children
follows viral exanthem or URI |
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What is the differential for acute ITP?
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aplastic aplasia
acute leukemia metastic tumor |
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How do you diagnose ITP?
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bone marrow examination
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Who gets chronic ITP?
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adults, age 20-50
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What is the differential for chronic ITP?
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SLE
primary hematologic disorder |
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People with ITP usually present with what?
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Mucosal or skin bleeding: epistaxis, oral bleeding, menorrhagia, purpura, petechiae
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True or False: Thrombocytosis, thrombocytopenia, and ITP can present with splenomegaly.
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False: Thrombocytosis and thrombocytopenia can both present with splenomegaly, but ITP does not.
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What labs would you expect with ITP?
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thrombocytopenia
mild anemia slightly enlarged platelets normal bone marrow normal coagulation studies |
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10% of ITP patients also have what?
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a coexistent hemolytic anemia (Evans syndrome)
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How do you treat ITP?
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Glucocorticoids
IV immunoglobulin Splenectomy Immunosuppressive drugs - azathioprine - cyclophosphamide - vincristine - vinblastine - cyclosporine Danazol |
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What is the definitive treatment for ITP?
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splenectomy
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If platelet count falls below 5,000, what is the patient at risk for?
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cerebral hemorrhage
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True or False: ITP with have normal bone marrow biopsy and normal coagulation studies.
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True.
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What deficiency causes TTP?
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deficiency of a von Wildebrant factor
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What causes TTP?
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pregnancy
metastatic cancer mitomycin C - antitumor antibiotic high-dose chemotherapy HIV infection Drugs - Ticlopidine - Estrogen |
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What are the clinical findings of TTP?
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Thrombocytopenia (pallor, purpura, petachiae)
Microangiopathic hemolytic anemia Renal failure Neurological abnormalities (confusion, delirium, seizures, hemiparesis, aphasia) Pancreatitis Involvement of myocardial vessels |
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What causes the sudden death in TTP patients?
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involvement of myocardial vessels
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What is the differential for TTP?
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DIC
Vasculitis SLE HUS Evans syndrome |
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What labs do you expect with TTP?
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increased indirect bilirubin
increased LDH negative Coombs' test increased reticulocytes fragmented RBCs (schistocytes, helmet cells) decreased platelets evevated firbrin degradation products proteinuria increased BUN |
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How do you treat TTP?
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large-volume plasmapheresis
- daily until patients are in complete remission |
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What causes hemolytic-uremic syndrome (HUS) in children?
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occurs after a diarrheal illness due to shigella, salmonella, E.coli, or viral
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What causes HUS in adults?
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estrogen use or postpartum
delayed complication of high-dose corticosteroids delayed complication of autologous bone marrow or stem cell transplantation use of cyclosporine or tacrolimus familial type |
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What are the clinical findings and laboratory findings of HUS?
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Microangiopathic hemolytic anemia
Thrombocytopenia Acute renal failure |
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What differentiates TTP from HUS?
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TTP has neurological symptoms
HUS does not |
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What is the most common inherited bleeding disorder?
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Von Willebrand's Disease
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True or False: Von Willebrand Factor facilitates platelet aggregation.
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False: It facilitates adhesion.
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What type of Von Willebrand's Disease is the most common? What is the problem?
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Type I: quantitative decrease in vWF
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What is Type II Von Willebrand's Disease?
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normal or near-normal levels of a dysfunctional protein
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Is Von Willebrand's disease a qualitative or quantitative platelet disorder?
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qualitative
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What are the clinical manifestations of Von Willebrand's Disease?
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mucosal bleeding
GI bleeding incisional bleeding exacerbation with aspirin |
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True or False: Most causes of Von Willebrand's Disease are very serious and require hospitalization.
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False: Most cases are mild.
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True or False: In pregnancy, bleeding due to Von Willebrand's Disease decreases.
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True.
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What lab findings would you see with Von Willebrand's Disease?
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prolonged bleeding time
Type I - vWF levels reduced Severe - prolonged PTT |
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What morphology would you expect to see with Von Willebrand's Disease?
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morphology normal
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What antigen measures immunologic presence of vWF?
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Factor VIII antigen
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How do you treat Von Willebrand's disease?
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factor VIII concentrates (during surgery or after major trauma)
oral contraceptives to suppress menses desmopressin (DDAVP) |
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How are the best candidates for desmopressin therapy?
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Type I Von Willebrand's disease patients.
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What antigen measures immunologic presence of vWF?
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Factor VIII antigen
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How do you treat Von Willebrand's disease?
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factor VIII concentrates (during surgery or after major trauma)
oral contraceptives to suppress menses desmopressin (DDAVP) |
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How are the best candidates for desmopressin therapy?
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Type I Von Willebrand's disease patients.
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What is the most common severe bleeding disorder?
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Hemophilia A
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Hemophilia A is a deficiency of what?
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factor VIII
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What are complications of Hemophilia A?
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necrosis of muscle - compartment syndrome
venous congestion - pseudophlebitis ischemic damage to nerves Repetative hemarthrosis -osteoarthritis -articular fibrosis -joint ankylosis -muscle atrophy Hematuria CNS bleeding |
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What labs would you see with Hemophilia A?
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prolonged PTT
normal PT, BT, fibrinogen level decreased factor VIII:C levels vWF normal |
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How do you treat Hemophilia A?
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Factor VIII concentrate (only with an episode)
Desmopressin for minor surgical procedures Infusion of factor VIII with E-aminocaproic acid before dental procedures |
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Hemophilia B is a deficiency of what?
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Factor IX
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How do you differentiate hemophilia A from B?
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They are clinically identical.
A: decreased factor VIII B: decreased factor IX |
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How do you treat hemophilia B?
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fresh-frozen plasma
factor IX concentrates |
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What is DIC?
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excess thrombin activity
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What causes DIC?
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Obstetric complications
Metastatic malignancy Massive trauma Bacterial sepsis Major hemolytic transfusion reactions |
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What is the most common finding of DIC?
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bleeding
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What are the lab findings in DIC?
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thrombocytopenia
decreased fibrinogen increased fibrin degradation products (positive D-dimer) prolonged PT prolonged PTT (sometimes) may have microangiopathic hemolytic anemia (RBC fragments and schistocytes) |
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How do you differentiate liver disease and DIC?
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liver disease has normal fibrinogen and normal or slightly low platelets
DIC has decreased fibrinogen and thrombocytopenia |
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How do you treat DIC?
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correct reversible causes
control the major symptom - bleeding - FFP - platelet transfusion - cryoprecipitate - thrombosis - IV heparin prophylaxis to prevent recurrence |
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What causes Vitamin K deficiency?
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Poor diet
Malabsorption Broad-spectrum antibiotics |
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What are the symptoms of Vitamin K deficiency?
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bleeding fom any site
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What lab findings would you see with Vitamin K deficiency?
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prolonged PT
prolonged PTT |
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How do you treat Vitamin K deficiency?
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subcutaneous vitamin K
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