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176 Cards in this Set
- Front
- Back
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which pathway does heparin affect?
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intrinsic pathway
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which pathway does warfarin affect?
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extrinsic
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defect in hemophilia A
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decreased factor VIII
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defect in hemophilia B
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decreased factor IX
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coag parameter prolonged in hemophilia?
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PTT
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what can be given in mild hemophilia A before minor surgical procedures?
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DDAVP, which can increase vWF which increases factor 8
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what is the most common hereditary bleeding disorder?
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von willebrand's disease
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how is von willebrand's inherited?
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autosomal dominant
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what is the role of vWF
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stabilizes factor 8 and enhances platelet aggregation/attachment to injured vascular endothelium
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how is bleeding time affected in hemophilia?
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it is normal ecause no platelet function abnormality exists
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what coagulation parameters are increased in von willebrand's?
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PTT and bleeding time
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how does heparin work?
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catalyzes activation of antithrombin III
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how does warfarin work?
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interferes with sythesis of vitamin K dependent cofactors
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what lab values are decreased in TTP?
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platelets and RBCs
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test that measures the ability of vWF to agglutinate platelets in the presence of ristocetin
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ristocetin cofactor assay
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treatment for major bleeding in pt with von willebrands
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FFP or cryoprecipitate
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what drug can be effective in mild von willebrand's
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DDAVP
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what is Evan's syndrome?
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ITP with autoimmune hemolytic anemia
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peak incidence of Evan's syndrome?
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20-50 years
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pathophys in ITP
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IgG autoantibody binds platelets, which causes lysis by splenic macrophages
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drug associated with ITP in adults?
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sulfa
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test for ITP?
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platelet-associated IgG test
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what does blood smear show in ITP
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megathrombocytes without schistocytes
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what should you consider before giving prednisone to a patient with ITP
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bone marrow aspiration because it can mask a leukemia
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most common inherited cause of hypercoagulability
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factor V leiden
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presence of blast cells
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acute leukemia
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most common leukemia in pts <15
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ALL
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most common leukemias in pts 15-59
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AML and CML
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most common leukemias in pts >60
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CLL
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ALL and philadelphia chromosome is associated with what type of prognosis
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unfavorable
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philadelphia chromosome
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t(9,22)
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what do blasts stain for in ALL
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PAS, TdT, CALLA
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what disorder is ALL associated with?
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down syndrome
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which type of AML is associated with DIC?
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M3
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which type of AML is associated with gingival hyperplasia?
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M5
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how does AML typically present?
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fatigue, hemorrhage, bruising
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patients with high WBC and AML may present with what?
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leukostasis, which arises from sludging of circulating leukemic blasts in tissue microvasculature
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what is pathognomonic for AML
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auer rods
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eosinophilic needle-like cytoplasmic inclusions
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auer rods, found in blast cells
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positive stains in AML
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sudan black and myeloperoxidase (10%)
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t(15,17)
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AML M3 - translocation involving retinoic receptor alpha and PML genes
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chemo for AML
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cytarabine plus daunorubicin
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what in addition to chemo is M3 type of AML treated with?
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all-trans-retinoic acid
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CLL is almost always a clonal malignancy of what type of cells?
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B cells
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median age of onset of CLL?
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65
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smudge cells
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CLL
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coexpression of CD19 and CD5 on flow
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CLL
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hypogammaglobulinemia is found in 50% of cases of what leukemia?
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CLL
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indications for chemo in CLL?
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progressive fatigue, symptomatic lymphadenopathy, or anemia/thrombocytopenia
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first-line therapy for CLL?
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fludarabine - avoid in hemolytic anemia
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what is Richter's syndrome?
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isolated LN transformation into aggressive large cell lymphoma (seen in CLL)
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approximately 90% of patients with CML have what?
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t(9,22)
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phases of CML
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chronic, accelerated, blast
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lab abnormalities in tumor lysis syndrome
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increased K, increased phos, increased uric acid, decreased Ca2+
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back pain, anemia, renal insufficency
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multiple myeloma
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what types of infections do pts with multiple myeloma tend to get?
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encapsulated organisms (strep pneumo, h flu)
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age distribution of Hodgkins
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bimodal; 20-30, then >50
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most common form of Hodgkin's
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nodular sclerosis - primarily affects young females
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free kappa and lambda chains on SPEP/UPEP
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Bence Jones proteinuria (multiple myeloma)
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xray findings in multiple myeloma
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lytic lesions, esp in axial skeleton, osteoporosis and fractures
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treatment for multiple myeloma
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few symptoms: thalidomide; chemo for pts who are refractory to thalidomide or who are acutely symptomatic
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M protein without evidence of systemic disease
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MGUS
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IgM spike, hyperviscosity, cold agglutinins
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waldenstroms macroglobulinemia
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spherocytes
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G6PD deficiency, membranopathy
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blister cell
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G6PD deficiency
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burr cell
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ARF, uremia
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heinz body
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thalassemia, hemoglobinopathies, enzymopathies
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schistocyte
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artificial heart valves and microangiopathic hemolytic anemias
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target cell
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splenectomy
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most common metabolic disorder of RBCs
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G6PD deficiency
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drugs that can precipitate hemolysis in pts with G6PD deficiency
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antimalarials, quinolones, sulfa drugs, NSAIDs, nitrofurantoin, fava beans, vitamin C
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decreased iron, increased TIBC, decreased ferritin
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iron deficiency anemia
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decreased iron, decreased TIBC, normal/increased ferritin
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anemia of chronic disease
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increased iron, normal to increased TIBC, increased ferritin in pt with microcytic anemia
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sideroblastic anemia
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treatment for sideroblastic anemia
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pyridoxine (vit B6)
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levels to check in pts with suspected thalassemia
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HbA2, HbF
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how does G6PD present
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jaundice, dark urine, ATN 1-3 days after exposure
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pentad of TTP
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FAT RN
fever anemia thrombocytopenia renal dysfunction neurologic abnormalities |
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acute chest syndrome
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chest pain, pulmonary infiltrate, increased WBC, hypoxia (indistinguishable from PNA)
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what is aplastic crisis often due to in sickle cell?
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parvovirus B19
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what is hemolytic crisis in sickle cell associated with?
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G6PD deficiency
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anemia, thrombocytopenia following mild viral illness or gastroenteritis with E. coli O157:H7
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HUS
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PT/PTT in HUS
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normal
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LDH level in HUS
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increased
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causes of TTP
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HIV, pregnancy, OCP use
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PT/PTT in TTP
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normal, but bleeding time is increased
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treatment for TTP
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large volume plasmapheresis, corticosteroids, ASA, splenectomy
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causes of DIC
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sepsis, transfusion reaction, neoplasia, trauma, obstetric complications (amniotic embolus and septic abortion)
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coag parameters affected in DIC
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increased PT/PTT, and bleeding time
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fibrinogen level in DIC
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decreased
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Howell-Jolly bodies
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cytoplasmic remnants of nuclear chromatin normally removed by spleen seen in sickle cell disease
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vaccines for patients with sickle cell
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h flu and pneumococcal
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penicillin prophylaxis for sickle cell patients under what age
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5
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defect in sickle cell
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defect in beta globulin chain
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defect in thalassemia
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decreased quantity of alpha or beta globulin chain
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defect in porphyria
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defect in heme synthesis
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what does electrophoresis show in thalassemia?
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increased HbF, decreased HbA, and increased HbA2
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treatment for alpha thal
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periodic transfusions
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treatment for B thal major
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aggressive transfusions, splenectomy to enhance RBC survival; BMT; ddeferoxamine
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treatment for B thal minor
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avoid oxidative stress
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secondary causes of polythycemia
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hypxia - COPD, lung disease, smoking, high altitude, RCC
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red face and pruritis after warm shower
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polycythemia
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epo level in polycythemia vera
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decreased
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what does p vera increase the risk of?
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conversion to CML, myelofibrosis, or AML
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most frequent cause of transfusion reaction
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ABO incompatibility due to clerical error
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what can hemoglobinuria associated with transfusion reaction lead to?
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ATN, olguria, renal failure
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what do patients with IgA deficiency have a reaction to with a transfusion
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donor IgA
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pathophys of acute transplant eaction
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cytotoxic T cells react against foreign MHCs
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how does GVHD often present
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painful pruritic maculopapular rash, hepatic dysfunction, diarrhea
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hypersegmented neutrophils
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folate/B12 deficiency
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basophilic stippling
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lead poisoning
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bite cells
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G6PD; hemolytic anemia
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teardrop-shaped RBCs
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myelofibrosis
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schistocytes, helmet cells, fragmented RBCs
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intravascular hemolysis
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acanthocytes and spur cells
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abetalipoproteinemia
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polychromasia
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from reticulocytosis; alerts to possibility of hemolysis
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rouleax formation
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multiple myeloma
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iron inclusions inside RBCs of bone marrow
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sideroblastic anemia
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extramedullary hematopoeisis
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thalassemia
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crew cut skull
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thalassemia
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eophageal webs, iron deficiency anemia, glossitis
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plummer vinson syndrome
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anticonvulsant causing folate deficiency
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classically phenytoin
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neurologic deficiencies in B12 deficiency
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loss of sensation and position sense, paresthesias, ataxia, spasticity, hyperreflexia, positive babinski, dementia
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polychromatophilic stippling
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sideroblastic anemia
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increased MCHC
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spherocytosis
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drugs known to cause aplastic anemia
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chloramphenicol, carbamazepine, phenylbutazone, sulfa drugs, zidovudine, gold
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what is myelophthisic anemia?
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space-occupying lesion in bone marrow
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blood product for IgA deficiency
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washed red blood cells
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what is the most common cancer in most organs?
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metastatic CA
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treatment for spinal cord compression?
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steroids, MRI, radiation
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edema and redness of neck, face and CNS symptoms
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SVC syndrome
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lung cancer associated with Cushing's
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small cell carcinoma
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lung cancer and PTHrP
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squamous cell
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lung cancer and SIADH
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small cell
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what is Eaton-Lambert syndrome?
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myasthenia gravis-like disease from lung cancer that spares ocular muscles
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lung cancer associated with Eaton-Lambert syndrome?
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small cell carcinoma
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most common histological type of breast cancer?
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invasive ductal carcinoma
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classic tumor marker for colon CA?
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CEA
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whipple's triad
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hypoglycemia (<50), CNS symptoms due to hypoglycemia, and improvement with administration of glucose
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what do glucagonomas cause
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hyperglycemia with high glucagon and migratory necrotizing skin erythema
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what do VIPomas cause?
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watery diarrhea, hypokalemia, achlorhydria
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most common type of ovarian CA
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serous cystadenoma
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ovarian tumor that causes virilization
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sertoli-leydig cell
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ovarian tumor that causes femininization and precocious puberty
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granulosa/theca-cell (classically present <30)
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what is Meigs' syndrome
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ovarian fibroma that causes ascites and right hydrothorax
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what cancers do OCPs reduce the risk of?
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ovarian and endometrial
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where does cervical cancer begin?
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transformation zone
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which is more common in alcoholics: folate or B12 deficiency
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folate
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what is associated with a poor prognosis in CLL?
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thrombocytopenia
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treatment for hairy cell leukemia
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cladribine
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treatement for CLL
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chlorambucin and prednisone
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treatment for non-Hodgkins
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CHOP
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testicular cancer associated wtih elevated AFP
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embryonal cell CA
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testicular cancer associated with elevated B-HCG
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choriocarcinoma
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with what complication is HIT associated?
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arterial thrombosis
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type of anemia caused by lead poisoning
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hypochromic, microcytic
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drugs that can cause folic acid deficiency
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phenytoin, TMP, MTX
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treatment for patient with familial colonic polyposis
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proctocolectomy at diagnosis
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treatment for pregnant patient with antiphospholipid antibody
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heparin and ASA
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cancers most likely to metastasize to brain
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lung, breast, melanoma
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what tumor should you suspect in child wtih intracranial calcifications on skull radiograph
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craniopharyngioma
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what tumor resembles a bunch of grapes coming out of the vagina
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sarcoma botryoids, type of embryonal rhabdomyosarcoma usually seen in kids
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who does nasopharyngeal cancer affect? what is is associated with?
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asians; EBV
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increased calcitonin and thyroid cancer
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medullary thyroid cancer
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most common primary tumor of the liver
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hemangioma
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benign liver tumor in women of reproductive age who take birth control pills
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hepatic adenoma
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liver cancer associated with IBD and liver flukes (clonorchis)
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cholangiosarcoma
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liver cancer and exposure to vinyl chloride
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angniosarcoma
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most common primary liver malignancy in children
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hepatoblastoma
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rash that doesn't respond to multiple treatments in an HIV+ patient
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Kaposi's sarcoma
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vascular skin tumor that commonly begins as a papule or plaque on the upper body or in the oral cavity; highly associated with HHV-8
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Kaposi's sarcoma
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how can you differentiate Wilms' tumor from neuroblastoma?
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neuroblastoma -adrenal gland, calcifications
wilms tumor- kidney, no calcifications |
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two major cytologic clues for histiocytosis
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CD1 positive cells and birbeck granules
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cytoplasmic inclusion bodies that look like tennis rackets
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birbeck granules
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expansile, lytic, well-demarcated benign lesion in proximal portion of the humerus in children and adolescents
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unicameral bone cyst
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how does retinoblastoma classically present?
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child less than 3 with leukocoria (red reflex changes to white) and/or unilateral exophthalmos
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