Salil Goorha acute leuk lecture 3/25/13 Flashcards
Define Leukemia
Group of heterogenous (different) diseases relating to accumulation of malignant myeloid derivatives (WBCs)
Why does Leukemia cause a problem
Bone Marrow screw ups lead to anemia, neutropenia, thrombocytopenia (low plateletts.
What classification system is used in AMerica?
FAB. based on morphology
What is the most common form of leukemia in children>
ALL—St. Jude
What is the distinction b/w primary and secondary AML?
Primary= De Novo
Secondary= develops from Myelodysplasia or something else
secondary is more difficult to treat.
Etiology (Causes) of Acute Leukemia?
Mostly idiopathic, some prior radiation and chemo, benzene exposure,myelodysplastic and myeloproliferative, downs, Fanconi’s anemia, aplastic and PNH
Where does malignant transformation arise in acute leukemia?
Hematopoeitic Stem Cell
Genetic damage leads to what three things:
Increased proliferation, lack of differentiation, lack of apoptosis
What does genetic damage in hematopoeitic stem cells culminate in? Something we can see under the microscope
Accumulation of blast cells…these are undifferentiated myeloid precursors.
What blast cell number defines leukemia?
Greater that 20% in bone marrow or peripheral blood.
How are lymphoblastic leukemias distinguished from myeloblastic?
Depends on whether the blasts are myeloblasts or lymphoblasts
How do you tell a myeloblast from a lymphoblast?
Do immunophenotyping (flow cytometry). Look at antigen expression.
Auer Rods are associated with what type of Acute Leukemia?
Acute myeloid leukemia
BIG: What immunological markers are associated with Myeloid
CD33, CD13, MPO, HLA-DR
BIG: What immunological markers are associated with Lymphoblastic Leukemia?
CD 10, CD 19, CD 20, TdT
Acute Promyelocytic Leukemia is associated with which translocation
t (15;17)
t (15;17) is associated with which gene fusion
PML-RARA.
t (8;21) gene fusion
ETO-AML1
What are the two core binding factor leukemias?
t(8;21) t/inv (16;16)
Poor risk is associated with how many chromosomal changes?
greater than 3
What cytogenetics are associated with good risk?
inv/t (16;16), t(8;21), t(15;17)
intermediate:
t(9;11)
Remission Therapy is what?
1 to 2 courses of intensive therapy to to achieve a complete response.
Post-remission
intensive short course therapy
Maintenance therapy
months to years of less intense therapy
AML is treated with what?
Chemo: Remission induction therapy, post-remission, maintenance
What drug is often used in treating older pts with AML
Azacitidine
Two divisions of ALL
T cell and B cell
What are the common genetic abnormalities in adults with ALL
MLL-AF4 and BCR-ABL…..Poor prognosis
Common genetic abnormality in children with ALL
E2A-PBX and TEL-AML…..good prognosis
What is the 3 phase treatment of ALL
Remission induction, Consolidation, Maintenance
What drug counters TUmor Lysis syndrome?
Allopurinol
