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94 Cards in this Set

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