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66 Cards in this Set
- Front
- Back
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What is the treatment for acute hemolytic transfusion reactions?
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-stop infusion
-maintain IV saline -treat hypotension with colloids/crystalloids -dopamine to support arterial BP, increase CO and renal vasodilation -furosemide, mannitol, lasix to induce diuresis -heparin for DIC -dialysis for renal failure |
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What are presenting signs/symptoms of hemolytic transfusion reactions?
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-fever
-chills -chest pain -hypotension -nausea -flushing -dyspnea -hemoglobinuria |
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What is a normal hemoglobin level? At what level do symptoms appear? At what level is transfusion likely indicated? What hemoglobin level is life threatening?
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- 12-16 g/dL = normal
- 10 g/dL = symptoms - 7 g/dL = transfusion indicated - 5 g/dL = life threatening |
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What is the most common adverse event associated with transfusion?
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circulatory overload
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What is the mechanism of transfusion related acute lung injury?
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-donor Ab (granulocyte, HLA) react with recipient white cells
-aggregates in lungs |
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What is the most common root cause of wrong unit transfusions? What is the chance of incompatibility?
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failed bedside clerical check (hung on wrong patient), 36% chance of incompatibility
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Which type of blood group antibodies are naturally occurring, directly agglutinate, fix complement, and cause intravascular hemolysis?
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IgM
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Which type of blood group antibodies are made in response to foreign antigen exposure, indirectly agglutinate, and cause extravascular hemolysis?
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IgG
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For a blood group B sample, what is the expected result of red cell test with anti-A antibodies? anti-B antibodies? What is the expected result of plasma test with A cells? B cells? What antibody is present?
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-red cell + anti-A: negative
-red cell + anti-B: positive -plasma + A cells: positive -plasma + B cells: negative -antibody present: anti-A |
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What antigen determines Rh positive or negative status?
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D antigen
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What is the leading cause of Hemolytic Disease of the Newborn?
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maternal anti-Rh Ab against fetal Rh Ag
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What antibody type is involved in major ABO incompatibility reactions? What antibody type is involved with other blood group antigens?
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-IgM causes ABO incompatibility reactions
-IgG causes other blood group antigen reactions |
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How are differentiation, apoptosis, and cell numbers affected in acute leukemias?
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-differentiation is blocked
-apoptosis is decreased -cell numbers may be up or down |
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How are differentiation, apoptosis, and cell numbers affected in myelodysplastic syndromes?
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-differentiation is abnormal
-apoptosis is increased -cell numbers are decreased |
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How are differentiation, apoptosis, and cell numbers affected in myeloproliferative syndromes?
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-differentiation is normal
-apoptosis is decreased -cell numbers are increased |
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What are the morphology, cytochemistry, and prognosis of M1 acute myelogenous leukemia?
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-morphology: basic myeloblasts
-cytochemistry: myeloperoxidase+ -prognosis: average |
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What are the morphology, cytochemistry, cytogenetics, and prognosis of M2 acute myelogenous leukemia?
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-morphology: myeloblasts with granules or Auer rods
-cytochemistry: myeloperoxidase+ -cytogenetics: t(8;21) -prognosis: average |
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What are the morphology, cytochemistry, cytogenetics, complications, treatment, and prognosis of M3 acute myelogenous leukemia?
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-morphology: promyelocytes with multiple Auer rods, bilobed nuclei
-cytochemistry: myeloperoxidase+ -cytogenetics: t(15;17) -complications: hemorrhage due to DIC -treatment: all-trans retinoic acid -prognosis: good if patient does not hemorrhage |
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What are the morphology, cytochemistry, and prognosis of M4 acute myelogenous leukemia?
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-morphology: myelomonocytic blasts
-cytochemistry: myeloperoxidase+, alpha-napthyl esterase+ -prognosis: extramedullary → bad |
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What are the differences between M4eo and M4 AML?
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-M4eo has better prognosis
-M4eo is caused by inversion of part of chromosome 16 |
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What are the morphology, cytochemistry, and prognosis of M5 acute myelogenous leukemia?
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-morphology: monoblasts
-cytochemistry: alpha-napthyl esterase+ -prognosis: extramedullary → bad |
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What are the morphology and cytochemistry of M6 acute myelogenous leukemia? What are alternate names?
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-morphology: megaloblastic erythroid cells, myeloblasts
-cytochemistry: periodic acid-Schiff+ -alternate names: erythroleukemia, DiGugliomo's syndrome |
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What morphology is associated with M7 acute myelogenous leukemia?
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-dysplastic megakaryocytes
-significant marrow fibrosis |
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What disorder is linked to t(8;21)? What gene product results? How is prognosis affected?
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-M2 acute myelogenous leukemia
-fusion of AML1 and ETO -prognosis is better |
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What disorders are linked to t(9;22)? What gene product results? How is prognosis affected? What treatment is required?
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-AML, CML, ALL
-fusion of BCR and ABL -prognosis is worse -treat with imatinib (Gleevec) |
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What disorder is linked to t(15;17)? What gene product results? How is prognosis affected? What treatment is required?
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-M3 acute promyelocytic leukemia
-fusion of PML and RARα -prognosis is better -treat with all-trans retinoic acid (Vesenoid) or arsenic trioxide (Trisenox) |
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What disorder is linked to inv16? What gene product results? How is prognosis affected?
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-M4eo acute myelomonocytic leukemia
-fusion of CBFβ and MYH11 -prognosis is better |
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What disorders are linked with 11q23 translocations? Which gene product is affected? What induces these translocations? How is prognosis affected?
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-AML, ALL
-MLL1 is fused with various partners -induced by topoisomerase II inhibitors (etoposide, doxorubicin) -prognosis is worse |
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What disorder is linked with FLT3 internal tandem duplication? How is the gene product affected? How is prognosis affected?
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-30% of acute myelogenous leukemias (typically M1, M2)
-FLT3 becomes constitutively active -prognosis is worse |
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What disorders are linked with 5q and 7q deletions? How is prognosis affected?
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-MDS, AML
-prognosis is worse |
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What morphology and cell types are associated with L1 "typical" acute lymphoblastic leukemia?
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-morphology: small blasts with scant cytoplasm
-cell types: pre-B and T cells |
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What morphology and cell types are associated with L2 acute lymphoblastic leukemia?
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-morphology: larger blasts, more irregular
-cell types: pre-B and T cells |
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What morphology, genetic anomaly, and cell type are associated with L3 acute lymphoblastic leukemia? What is another name for this disorder?
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-morphology: blasts with abundant vacuoles
-cytogenetics: t(8;14) c-myc gene -cell types: B cells -equivalent to Burkitt's lymphoma |
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Which karyotype is associated with better prognosis in acute lymphoblastic leukemia?
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hyperdiploidy (>50 chromosomes)
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Which disorder is linked with t(8;14)? How is the gene product affected? How is the prognosis affected?
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-L3 acute lymphoblastic leukemia/Burkitt's lymphoma
-c-myc gene fused downstream of lymphoid specific promoters → constitutively active -prognosis is controversial whether better or worse |
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Which lymphoid cell type is the most common in adult ALL? Which surface markers are present on it? What complications are associated with it?
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-pre-B cell
-CD10, CD19, TdT -CNS complications |
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Which surface markers are associated with T cell lymphoblastic leukemia? What complications are associated with it?
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-CD7, CD3, TdT, CD4, CD8
-hypercalcemia, mediastinal masses, CNS disease |
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Which blood disorders are associated with Down Syndrome?
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-20x risk of acute leukemia (ALL, AML - esp. M7)
-transient abnormal myeloproliferative disorder |
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Which type of leukemia is associated with Fanconi's anemia?
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10-40% incidence of AMML
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Which blood disorders are associated with Kostmann Syndrome?
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AML, MDS
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Which blood disorders are associated with ataxia-telangiectasia?
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ALL, lymphoma
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Which type of leukemia is associated with Li-Fraumini Syndrome?
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childhood ALL
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Which environmental exposures increase the risk of developing AML?
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-ionizing radiation
-benzene -agricultural exposures (pesticides, herbicides, etc.) -alkylating chemotherapy agents -topoisomerase inhibitors |
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Which hematologic disorders have a risk of transforming to AML?
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-myeloproliferative disorders
-paroxysmal nocturnal hemoglobinuria -myelodisplastic diseases |
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What number of leukemia cells is the lower limit of detection in bone marrow? How many are needed to cause symptoms? How many to cause death?
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-10^6 - 10^8 → lower limit of detection
-10^9 → symptoms -10^12 → death |
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What tests are involved in the evaluation of leukemia?
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1. peripheral blood smear
2. flow cytometry 3. bone marrow cytogenetics 4. southern blot (ALL) or cytochemical staining (AML) |
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How can WBC count, age, sex, race, and physical exam results help to determine the prognosis of ALL in a patient?
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-WBC count: worse if > 30,000/mcL
-age: worse if > 15 -sex: worse if male -race: worse if non-caucasian -physical exam: worse if CNS disease present |
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What drugs are used in induction chemotherapy of AML?
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-daunorubicin
-cytarabine (M3, M7) -add all trans retinoic acid for M3 -add imatinib for t(9;22) |
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What drugs are used in consolidation chemotherapy of AML?
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-daunorubicin + cytarabine
-or high-dose cytarabine |
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Which type of AML may require maintenance chemotherapy? What is the regimen?
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-M3 may require maintenance
-all trans retinoic acid -6 mercaptopurine -methotrexate |
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What drugs are used in induction chemotherapy of ALL?
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-vincristine
-prednisone -(usually) an anthracycline, L-asparaginase -add imatinib for t(9;22) |
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What is the consolidation chemotherapy regimen of ALL?
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CNS prophylaxis with intrathecal methotrexate
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What is the maintenance chemotherapy regimen for ALL?
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1-2 years of oral maintenance with:
-6 mercaptopurine -methotrexate -sometimes other agents |
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What is the ultimate cure rate for AML? M3? ALL?
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-AML: 40%
-M3: 80% -ALL: 20-30% |
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What are the five major kinds of myeloproliferative disorders?
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-chronic myelogenous leukemia
-polycythemia rubra vera -essential thrombocytopenia -myelofibrosis -hypereosinophilic syndrome |
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Which translocation is both necessary and sufficient to produce CML?
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t(9;22)
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What clinical features are present in CML?
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-splenomegaly
elevated WBC: -predominately granulocytes -intermediate-aged myeloid cells, basophils, and/or eosinophils |
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What screening test can be used in the diagnosis of CML?
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leukocyte alkaline phosphatase (LAP) score
-normal = high enzyme -CML = low enzyme |
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How is CML diagnosed?
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demonstration of t(9;22) by FISH (peripheral blood) or cytogenetics (bone marrow)
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What condition should be considered in the differential diagnosis of CML?
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leukemoid reactions caused by infection, malignancy, or after administration of cytokines (esp. G-CSF or GM-CSF)
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How is CML treated in the chronic phase? How effective is the standard treatment?
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-imatinib (Gleevec)
-100% clinical remission -70% cytogenetic remission -10-20% molecular remission |
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How is CML treated in the blast transformation phase?
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-acute leukemia therapy
-imatinib/dasatinib |
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What is the chromosomal/molecular defect that is associated with polycythemia rubra vera?
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V619F mutation of JAK2 tyrosine kinase → autonomous
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What clinical features are associated with polycythemia rubra vera?
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-high hemoglobin/hematocrit → hyperviscosity
-generalized pruritis, esp. after showers -splenomegaly |
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How is polycythemia rubra vera diagnosed?
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-elevated RBC mass (using radiolabeling)
-low erythropoietin -demonstration of V619F JAK2 mutation -panmyelosis (elevation of WBC and platelets too) |
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What conditions may present in a similar fashion to polycythemia rubra vera?
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-stress erythrocytosis (hot weather + middle age smoking men who take diuretics → reduced plasma volume, normal RBCs)
-secondary erythrocytosis (high epo) |
