Acute and Chronic Leukemias-Usera Flashcards
What are the 2 types of myelogenous leukemias?
Acute myelogenous leukemia (AML)
Chronic myelogenous leukemia (CML)
What are the four types of Lymphocytic/lymphoblastic leukemias?
Acute Lymphoblastic Leukemia (ALL)
Chronic Lymphocytic Leukemia (CLL)
Hairy Cell Leukemia
Adult T cell Leukemia
What are the 2 types of acute lymphoblastic leukemia (ALL)?
B cell and T cell
What is HTLV-1?
a type of adult T cell Leukemia
What kind of leukemia is prevalent in 0-14 year olds?
ALL
What kind of leukemia is prevalent in 15-59 year olds?
AML
What kind of leukemia is prevalent in 40-60 year olds?
AML/CML
What kind of leukemia is prevalent in elderly adults (greater than 60)?
CLL
What is this:
maturational arrest in granulocyte development, leading to proliferation of malignant precursor cells
Acute myelogenous leukemia
What age groups get acute myelogenous leukemia?
young to middle aged adults
APL acute myeloid leukemia has a recurrent genetic abnormality, what is it?
t(15;17)(q22;q12); PML-RARA
What AML are not otherwise specified?
AML with maturation
Acute monoblastic/monocytic leukaemia
What are the clinical features of AML?
anemia thrombocytopenia neutropenia leukocytosis with increased blasts Evidence of marrow failure
What are the signs/symptoms of AML?
fatigue
bleeding/ecchymoses
DIC (APL)
gum infiltration (monocytic)
If you have a marrow failures, what will you see?
marrow packed with blasts
What do you do with an aspirate?
flow cytometry smear cytogenetics (FISH, Karyotype)
In AML you are going to see (blank), the features of these are fine powdery chromatin, they are immature cells with widely dispersed chromatin with high nuclear cytoplasmic ratio.
blasts
The only morphologic key when dealing with blasts is (blank). They tell you if they are myeloid cells. (cuz they are fused primary granules)
auer rods
Acute promyelocytic leukemia corresponds to (blank).
FAB M3
APML with t(blank), q(blank)
15;17
22;12
Acute promyelocytic leukemia is found in (blank) percent of AML
5-8
What age group gets promyelocytic leukemia?
any age, but usually 3-5 decades of life
What does APML typically present with?
DIC
APML often has large numbers of (blank)
auer rods
IF you see tons and tons of auer rods what should you be thinking of?
promyelocytic leukemia
What is the pathogenesis behind the APL translocation?
balanced translocation b/w RARA and PML that results in a fusion product that arrests the cell at the maturational stage so it cant progress from promyelocyte to myelocyte.
What is RARA and what does this mean for APL?
it is a retinoid acid receptor alpha (receptor for vit A) so this means if you get APL you can cure it by taking vit A and an alkylating agent
APL has a translocation between chromosome (blank) and (Blank)
15 and 17
How do you treat acute promyelocytic leukemia?
ATRA and alkylating agent
80-90% remission
What is this:
auer rods are rare
gum infiltration is frequently the presenting sign
Acute monocytic leukemia
What is this:
15% of adult leukemias
may be related to radiation or benzene
pluripotent
t (9,22) BCR-ABL fusion gene AKA philadelphia chromosome
Protracted chronic phsae, sometimes many years, followed by blast crisis
Chronic myelogenous leukemia
What are the chromosomes involved in chronic myelogenous leukemia?
9 and 22
What is the are the fusion genes associated with CML?
BCR-ABL fusion gene AKA phildelphia chromosome
Chronic myelogenous leukemia has a (blank) chronic phase, sometimes many years, followed by blast crisis
protracted
Philadelphia chromosome is caused by a (blank) mutation
de novo
Is the philadelphia chromosome specific for CML?
no
(blank) fusion gene produces a constitutively active tyrosine kinase, leading to dysregulation of proliferation and normal apoptosis.
BCR-ABL1
The BCR-ABL1 fusion product primarily deals with the (blank) pathway
STAT5 pathway
In chronic myelogenous leukemia, the WBC count is greater than (blank)
5X10^9
What is this: splenomegaly bleeding/ecchymoses fever, night sweats, weight loss less than 10% blasts Characteristic diffusion, with full spectrum of granulocytes represented
Chronic Myelogenous Leukemia
Why do you get splenomegaly in CML?
deposition of messed up neutrophils
(blank) is the terminal stage of chronic myelogenous leukemia that is characterized by a marked increase in the proportion of blast cells, by fever and pain in the bones, and by increased severity of anemia, thrombocytopenia, and splenomegaly—called also blastic crisis
Blast phase (crisis)
What is this: progression to acute leukemia greater than 20% blasts large aggregates in BM and extramedullary tissue Usually fatal
Blast phase (crisis)
CML-> deposition of malignant cells in his tissues. (blank) is when CML has reached blast breaking point and then wil go into acute ML. Rapidly fatal. (blast count above 20% makes chronic turn to acute)
Blast crisis
How do you treat CML?
with tyrosine kinase inhibitors (imatinib, gleevec)
and allogenic stem cell transplant
In CML, allogenic stem cell transplants, give you a 5 year progression free survival in (blank) percent of patients
80-95%
What is the most common leukemia in children?
B cell acute lymphoblastic leukemia
Which is more prevalent B cell acute lymphoblastic leukemia or T cell acute lymphoblastic leukemia?
B cell (80-85%)
(blank) percent of B cell acute lymphoblastic leukemia involve children under 6 years of age.
75%
What will you see in acute lymphoblastic leukemia?
Primitive, undifferentiated lymphoblasts, express CD10 and tdt!!!
What are the clinical features of acute lymphoblastic leukemia?
marrow failure: anemia, thrombocytopenia, neutropenia. WBC count can be variable.
EXTRAMEDULLARY involvement common in CNS, LN, Spleen, liver, testis
What is the prognosis of acute lymphoblastic leukemia?
90-95% complete remission
What are favorable factors associated with acute lymphoblastic leukemia?
age 1-10
t (12,22) ETV-RUN X1
hyperdiploidy (greater than 52 chromosomes)
What are the chromosomes involved with acute lymphoblastic leukemia?
t(12;22)/ETV-RUNX1
Once you realize someone has acute lymphoblastic leukemia, what should you immediately look at?
CNS
What does T cell acute lymphoblastic leukemia typically present with?
mediastinal mass or acute leukemia
What percent of childhood leukemias are T-cell ALL?
15-20%
What are the T cell markers for acute lymphoblastic leukemia?
CD10-, tdt+
might see CD3,CD4, and CD8 on one cell
(blank) is a pan T cell marker.
CD3
Chronic lymphocytic leukemia is a clonal (blank) cell neoplasm
B
Chronic lymphocytic leukemia is a clonal B cell neoplasm that is (blank) chain restricted.
light
kappa vs lambda
What is the most common human leukemia?
chronic lymphocytic leukemia
What is the most common leukemia in ederly?
Chronic lymphocytic leukemia
Which cells does chronic lymphocytic leukemia effect?
mature, virgin B-cells (CD19+,CD20+) blasts less than 10%
Where do you see aberrant co-expression of CD5 and CD23?
chronic lymphocytic leukemia
What is this: WBC 10-100 x 109/L Small mature lymphs Clumped chromatin Smudge cells
chronic lymphocytic leukemia (CLL)
Chronic lymphocytic leukemia is a (blank) disease so treatment is usually withhheld, however if you need treament then use (blank)
Indolent disease
chlorambucil
What is the prognosis of chronic lymphocytic leukemia?
variable
What makes you have a favorable prognosis (median survival 293 months) with CLL?
mutated IGVH
What makes you have an unfavorable prognosis with CLL?
ZAP-70 and CD38 expression
2-8% percent of CLL patients develop (blank) with a median survival of less than 1 year
DLBCL (richtor transformation)
What is this: Neoplasm of memory B cells Older Caucasian males Indolent disease Pancytopenia, monocytopenia* Splenomegaly CD11c, CD25, CD103
hairy cell leukemia
What are the markers associated with hairy cell leukemia?
CD11c, CD25, CD103
(only time you will ever see a three digit marker)!!!
What is this:
Frequent splenic red pulp sequestration – splenomegaly
Diffuse bone marrow involvement – fried egg appearance
Dense reticulin network – “dry tap”
Hairy cell leukemia
What is this:
CD4+ T cell neoplasm caused by HTLV-1
LA, hepatosplenomegaly, lymphocytosis
Rapidly progressive and fatal within 1 yr
Adult T cell leukemia
Where is adult T cell leukemia endemic?
Japan
West Africa
Caribbean