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94 Cards in this Set
- Front
- Back
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What is classification (FAB) of AML based on?
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Blast morphology
Surface antigens (specific for each cell type) Histochemical stains |
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What percentage of leukemia cases are AML in adults and in children?
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Adults = 50%
Children = 20% |
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Which more commonly occurs as a secondary malignancy, ALL or AML?
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AML
(are often fatal) |
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Are blasts always found in the peripheral blood of a patient with AML?
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No
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What will the total white count be (low, high, etc.) in a patient presenting with AML?
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It can be elevated, normal, or low
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Is DIC commonly associated with AML or ALL?
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AML
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Blasts in AML will often contain what?
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Auer Rods
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Small blasts (peripheral) with very high nuclear to cytoplasmic ratios and inconspicuous nucleoli are typical of what?
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Childhood ALL
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In patients with acute leukemia, what percentage of the nucleated bone marrow cells are blasts?
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20-30%
(WHO classification requires >20%) |
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Are cytochemical tests important in classifying AML?
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Yes
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What cytochemical tests are typically used in the classification of AML?
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MPO (myelopoeroxidase)
CAE (chloroacetate esterase) ANAE (alpha naphthyl acetate esterase) |
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What are the cytogenetic abnormalaties commonly seen in AML? Designate prognosis of each as good or poor.
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t(15;17) [acute promyelocytic leukemia] - good
t(8;21) - good t(16;16) - good -5 - poor -7 - poor |
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What percentage of AML patients have overt CNS disease?
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10-15%
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What is the typical duration of chemotherapy for AML? (non-M3)
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6-12 months (mostly inpatient)
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What is the standard treatment for M3 AML?
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ATRA (all-trans-retinoic acid)
Less-invasive chemotherapy |
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How is remission defined in AML?
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<5% blasts in marrow
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About 80% of ALL cases are positive for what lymphoid surface marker?
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CALLA
(common acute lymphoblastic leukemia antigen - a marker of pre-B cells) |
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What percentage of ALL cases are positive for T-cell markers?
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~15%
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What is the age incidence peak of ALL?
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2-10 years
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What is the most common malignancy of childhood?
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ALL
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Which has a higher incidence of CNS disease, AML or ALL?
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ALL
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Flow cytometry can be used to classify ALL into what three subtypes?
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Precursor B-cell
Mature B-cell Precursor T-cell |
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Which is the most common phenotype of ALL in children and what is its relative prognosis?
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Precursor B-cell
Has the best prognosis in children |
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Which phenotype of ALL has the best prognosis in adults?
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Precursor T-cell
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Precursor T-cell ALL is characteristically positive for what antigens?
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CD3
CD4 CD8 TdT CD34 |
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Which type of ALL has a predilection for teenage and young adult males?
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Precursor T-cell
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How do patients with precursor T-cell often present? (unique to this phenotype)
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Mediastinal mass
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Mature B-cell ALL is often associated with which FAB classification?
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L3
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Precursor B-cell ALL is positive for what surface immunoglobulins?
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None - cells are surface immunoglobulin negative
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Which phenotype of ALL is surface immunoglobin positive and light chain restrictive?
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Mature B-cell
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The FAB L3 morphology of ALL is classically associated with what? (3)
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Blue cytoplasm
Prominent cytoplasmic vacuoles Abnormalities of the c-myc oncogene |
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FAB L3 leukemia is closely associated with what?
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Burkitt's lymphoma
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The differential diagnosis of childhood ALL includes what? (3)
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Idiopathic thrombocytopenic purpura (ITP)
Aplastic anemia Reactive lymphocytoses |
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A bone marrow aspirate of a child with ITP will show what?
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Normal red and white blood cell precursors with increased numbers of megakaryocytes
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What flow cytometry finding readily distinguishes reactive lymphocytes from those found in ALL?
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Predominance of mature T-cells
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What are the common cytogenetic abnormalaties found in ALL? Indicate the prognosis of each.
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Hyperploidy (50 or more chromosomes) - favorable
t(9;22) (Philly chromosome) - unfavorable t(8;14) - unfavorable t(4;11) - unfavorable Hypoploidy - unfavorable |
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How does typical chemotherapy treatment for ALL compare to that for AML?
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Less intensive
Longer duration (2-3 years) Usually outpatient |
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Which has a higher remission rate, ALL or AML?
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ALL
(95% at day 28 of therapy) |
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What are the two most important prognostic factors for ALL?
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Age (2-10 years optimal)
Initial white count (<10,000 optimal) |
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When is bone marrow transplant typically used in the treatment of ALL?
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Sometimes for patients with relapsed ALL
Sometimes as "up-front" therapy for patients with high risk features in first remission |
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What is the most common leukemia?
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CLL (25-30% of all leukemias)
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What is the median age at diagnosis for CLL?
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55 years
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Exposure to what is a risk factor for CLL?
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Agent orange
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What physical findings differ in the presentations of CML and CLL?
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Hepatomegaly is common in CML but not so in CLL
Lymphadenopathy is common in CLL but rare in CML Splenomegaly commonly seen in both |
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Autoimmune hemolytic anemia occurs in 10-25% of cases of what leukemia?
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CLL
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CLL is associated with what three disease transformations?
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Prolymphocytic leukemia
Diffuse large B-cell non-Hodgkin lymphoma Hodgkin lymphoma |
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Increased expression of what protein confers a survival advantage to the malignant clone in CLL?
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Bcl-2
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Give the stages (5) of CLL and describe each.
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Stage 0: lymphocytosis only
Stage 1: lymphocytosis and lymphadenopathy Stage 2: lymphocytosis and splenomegaly Stage 3: lymphocytosis and anemia Stage 4: lymphocytosis and thrombocytopenia |
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What are cytogenetic abnormalaties commonly seen in CLL? (4)
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13q deletion
Trisomy 12 11p deletion (ATM deletion) 17p deletion (p53 deletion) |
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Is lack of somatic hypermutation of the variable region of IgH in cases of CLL associated with a good or bad prognosis?
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BAD
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In cases of CLL, is Zap-70 expression associated with a better or worse prognosis?
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Worse
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What is the treatment for asymptomatic CLL?
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None - treatment confers no survival advantage
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Are CML and CLL more common in males or females?
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Males
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Where (cell type) does the transforming event occur in CML?
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Hematopoietic pluripotent stem cell (HSC)
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Where (cell type) does the transforming event occur in CLL?
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Mature lymphocyte
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The Philly chromosome is most commonly associated with and necessary for which leukemia?
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CML
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CML pluripotent stem cells posses what properties that lead to expression of the disease? (3)
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1. The capacity for self renewal
2. The ability to differentiate along the granulocytic pathway 3. An increased proliferative rate and increased apoptotic threshold (conferred by BCR-ABL) |
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What is the translocation on the Philly chromosome?
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t(9;22)
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What are the three phases of CML?
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Chronic (<10% blasts)
Accelerated (10-29% blasts) Blast (>30% blasts) |
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Once CML reaches blast crises, does it more commonly take the form of ALL or AML?
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AML (66-75% of cases)
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How is major cytogenetic remission of CML defined?
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<35% of cells on cytogenetics are + for t(9;22)
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Disease free survival after sibling matched BMT for chronic phase CML is what? (%)
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45-70%
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What is the mechanism of imatinib (Gleevec)?
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Inhibits BCR-ABL kinase function
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What is the most common cause of enlarged lymph nodes?
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Benign reactive lymphadenopathy
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Follicular hyperplasia is a proliferation of what?
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B lymphocytes
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Paracortial hyperplasia is a proliferation of what?
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T lymphocytes
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Are tingible-body macrophages seen in follicular or proliferative hyperplasia?
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Follicular
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Sinus histiocytosis is a proliferation of what?
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Macrophages
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Which morphological subtypes of Hodgkin's lymphoma have good prognoses?
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Nodular sclerosis
Lymphocyte predominant |
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Which morphological subtype of Hodgkin's lymphoma has the worst prognosis?
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Lymphocyte depleted
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Are non-Hodgkin's lymphomas stem cell disorders?
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NO
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What is essential for the growth of myeloma cells?
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IL-6
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What is the 5 year survival of myeloma? (%)
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30%
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What are the common clinical manifestations of multiple myeloma? (5)
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Bony pain (70%)
Anemia (60%) Hypercalcemia (20%) Renal failure (20%) Infections |
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What has been the standard of care for pts with multiple myeloma who are not transplant candidates?
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Steroids
Oral alkylating agents (MP) |
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What is the IV induction therapy for transplant in multiple myeloma?
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"VAD"
Vincristine Adriamycin Dexamethasone |
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What is the oral induction therapy for transplant in multiple myeloma?
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"TD"
Thalidomide Dexamethasone |
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What is given as adjunctive care to pts with multiple myeloma?
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Bisphosphonates
Epo G-CSF Antibiotics |
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If there is a monoclonal antibody found on immunofixation, what further finding would indicate monoclonal gammopathy over MM?
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Protein/albumin discordance
(protein > 2x albumin) |
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MGUS is related to infection with what?
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H. pylori
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What are the three things listed for dx of amyloidosis?
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Rectal biopsy
Supcutaneous fat aspiration Examine under polarized light, stain with Congo red |
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Which type of biopsy is the best option in the workup of lymphoma?
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Excisional
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What is LASSS?
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> normal LDH
Age > 60 Stage III or IV > 1 extranodal site Performance status ≥ 2 |
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Do the tumor cells appear more mature in FSCCL or DLCL?
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FSCCL
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What translocation is found in 85% of cases of FSCCL?
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t(14;18)
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What is the most common type of NHL?
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DLCL
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What are major risk factors for DLCL?
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HIV (60x)
Age SLE (3.6x) |
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Where are BCL2 and BCL6 located?
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BCL2 = 18q21
BCL6 = 3p27 |
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What is the treatment for DLCL?
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R-CHOP
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What is the cure rate for DLCL?
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20-80%
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What Ig type do Burkitt's cells express?
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IgM
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What gene product is overexpressed in Burkitt's?
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Myc
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Where is Myc located?
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Chromosome 8
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What is the "starry sky" histological appearance associated with?
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Burkitt's lymphoma
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