Schowinsky ALL and AML Flashcards
What are acute leukemias
clonal neoplastic hematopoietic cells, usually immature, presenting as a rapidly progessing disease
define AML
leukemic cells resembling cells of myeloid lineages
define ALL
leukemic cells resembling precursor lymphocytes
risk factors for acute leukemia
previous chemotherapy, exposure of active marrow to ionizing radiation, tobacco smoke, benzene exposure, and genetic syndromes
T or F: the majority of acute leukemias occur in the apparent absence of risk factors
TRUE
T or F: Presenting signs and symptoms usually result from replacement of normal marrow cells by leukemic cells.
True: can exhibit S/sx of anemia, thrombocytopenia, and neutropenia
T or F: Thrombotic events, DIC, and direct infiltration of skin, gums, and lymph nodes are very common presenting signs and symptoms of acute leukemias
False: these do occur, but more rarely
T or F: neoplams of precursor lymphoid cells manifest as AML, and commonly have a solid mass (lymphoblastic lymphoma)
False: manifest as ALL, and solid masses are less common
T or F: 75% of cases of ALL occur in children less than 6 yrs old
TRUE
name 3 factors to notice when diagnosing ALL
1) nearly always see “packed marrow” full of blast cells. 2) peripheral WBC count can vary greatly (high, normal, or low) 3) determine the blast type by immunophenotyping
what is the basic marker of immaturity
CD34
common lymphoblast marker
TdT
basic markers for precursor-B cells
CD19, CD22
basic markers for precursor-T cells
CD3, CD7
T or F: B-ALL accounts for 80-85% of cases of ALL
TRUE
T or F: B-ALL usually lacks mature B cell markers like CD20 and surface immunoglobulin
TRUE
T or F: B-ALL is the typical ALL of adulthood
FALSE
What does t(9:22) indicate
“Ph+ ALL”. B-ALL with Philadelphia chromosome, produces a fusion protein of BCR-ABL. Seen in 25% of adult ALL and 2% of childhood ALL. WORST PROGNOSIS OF ANY SUBTYPE ALL
What does translocation 11q23 indicate
“MLL” B-ALL more frequently seen in neonates and young infants. Has a poor prognosis
What does t(12:21) indicate
“ETV6-RUNX1” 25% of cases of childhood B-ALL. Very favorable prognosis
T or F: T-ALL accounts for around 25% of cases of ALL
TRUE
T or F: At least 105% of ALL cases are B-ALL and T-ALL
FACT!!! 80% B-ALL + 25% T-ALL = 105 fucking %! Where are your gods now?!
T or F: T-ALL is less frequent in adolescents and young adults.
FALSE
T or F: T-ALL often presents with a T-lymphoblastic lymphoma.
TRUE: T-LBL can often present as a mediastinal mass
What can you say about T-ALL when it comes to WBC and sex?
markedly elevated WBC count compared to B-ALL, favors males over females
T or F: In general, ALL has a “good prognosis” in children and a worse prognosis in adults
TRUE: complete remission rates are >95% vs 60-80% and cure rates are at 80% vs <50% in kids and adults respectively
Give 2 factors of better prognosis in ALL with reference to age and disease
school age (2-10 yo), B-lymphoblastic hyperdiploidy (51-65)
Give some factors of worse prognosis in ALL with reference to age and disease
Infants (10 yo), Very high WBC, T-lymphoblastic hypodiploidy (<46), slow response to Rx, Min. Residual disease
T or F: AML is a more heterogeneous disease than ALL and is typically seen in adults
TRUE
What is the big marker for diagnosing AML for most cases?
> 20% myeloblasts in the marrow and/or peripheral blood
basic markers for the common myeloid lineage
CD117(C-kit) and myeloperoxidase
What is one hystological feature that can help identify myeloblasts sometimes?
Auer rods
T or F: some cytogenetic abnormalities allow for diagnosis of AML regardless of blast count
TRUE
What does t(8:21) indicate
“RUNX1-RUNX1T1” 5% of AML cases, younger patients, “AML with maturation” some mature neutrophil production occurs, relatively good prognosis, diagnostic regardless of blast count
What does inv(16) or t(16:16)
“CBFB-MYH11” 5-10% of AML cases, younger patients, “baso eso” abnormality, typically have myelomonocytic leukemia (myeloblasts and monocytes), relatively good prognosis
What does t(15:17) indicate
“PML-RARA” acute promyelocytic leukemia (APL), also called M3-AML, hypergranular morphology resembling normal promyelocytes, may see multiple Auer rods, diagnostic of AML regardless of count. Good prognosis if caught early
what does the RARA gene do?
it is a retinoic acid receptor. PML-RARA is a poorly functioning retinoic acid receptor. Proper function required for differentiation past promyelocyte stage
How do we treat APL?
supraphysiologic doses of all-trans retinoic acid (ATRA), do not require traditional induction chemo. Can be treated with ATRA and arsenic salts (little morbidity and almost no mortality)
What is something really bad that is associated with APL?
DIC
what does t(1:22) indicate?
“RBM15-MKL1”, Megakaryoblastic differentiation, seen in infants with Down syndrome, relatively good prognosis
What does 11q23 indicate?
“MLL” multiple possible partner genes, show some degree of monocytic differentiation, POOR prognosis
Describe therapy related AML (t-AML)
AML arising secondary to DNA damage from a prior treatment, 10-20% of AML, VERY BAD PROGNOSIS
AML, Not Otherwise Specified (NOS)
cases lacking recurrent cytogenetic findings, no known therapy, defined by morphology and immunophenotyping
What subtype of AML NOS has myeloblast cells
Undifferentiated, minimally differentiated or with maturation
What subtype of AML NOS has myeloblast and monocyte cells
Myelomonocytic
What subtype of AML NOS has monoblast, promonocyte, and/or monocyte cells
monoblastic or monocytic
What is seen with monoblastic or monocytic AML NOS
Leukemic cells often causes lesions in skin and gums
What subtype of AML NOS has Megakaryoblasts cells
Megakaryoblastic
What is seen with Megakaryoblastic AML NOS
often associated with significant marrow fibrosis
What molecular abnormality has poor prognosis
FTL3 Internal Tandem Duplication (ITD)
What 2 molecular abnormalities have a good prognosis
Nucleophosmin-1 mutation (NPM1) and CEBPA Mutation. This is assuming there is no FLT3 ITD
T or F: patients that are healthy enough can have hematopoietic stem cell transplant (SCT).
TRUE: Therapy of choice for younger patients, high risk disease, and relapsed disease
