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66 Cards in this Set

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