Acute Leukemia Flashcards
What is the global picture idea to acute leukemia?
There is a disruption in the step wise fashion in maturing blood cells. This causes a build up of stem cells or the blast cells.
How do we define acute leukemia?
Accumulation of greater than 20% of blasts.
What is the big picture MOA of disruption by the accumulation?
The overwhelming number of blasts hinder normal hematopoietic. So clinically, it can present as anemia/fatigue if RBCs aren’t being produced, thrombocytopenia/bleeding if platelets arent being produced, and neutropenia/infection.
What do all the blasts do once they build up? What are three identifying features of the blast?
They will pour out into the blood causing a high WBC count.
Large, immature, and a punched out nucleolus.
What are the two types of acute leukemia?
Lymphoblastic and myelogenous
What is a good definition of acute lymphoblastic leukemia and what is the marker we use to identify these?
Neoplastic accumulation of lymphoblasts, greater than 20%, in the bone marrow.
TdT, which is a DNA polymerase only found in the nucleus of lymphoblasts
ALL classically presents in what patient population and what is a high yield association with ALL, but what is the catch?
Children
Down syndrome, but will not arise typically until after 5 years of age.
What are the two subtypes of ALL, which one is most common, and are the three markers for the most common one?
B ALL and T ALL
Most common is B
CD 10,19,20
Explain the treatment of B ALL and what two things must we make sure to do?
Excellent response to chemotherapy.
Need prophylaxis or independent or custom treatment to testicles and CSF because of their blood barriers
Explain the concept of prognosis of B ALL being based on cytogenic abnormalities?
Translocation of 12:21 has good prognosis and more seen in kids
Translocation of 9:22 has a poor prognosis more commonly seen in adults
What are the markers for T All?
CD2-8
They do not express cd10
How does T ALL usually present and what is the other name for it?
Teenager with a thymic mass
Acute lymphoblastic lymphoma
What is the marker for acute myeloid leukemia, how do we identify the marker, and what patient population do we typically see it in?
MPO
Under microscope, there will be crystal aggregates of MPO seen as Auer rods.
50-60
Explain how we subclassify AML?
We start with trying to see if there are any cytogenic abnormalities. If there are, we subclassify it by that
If not, then we subclassify it by the lineage of the immature myeloid cell.
What is the high yield subtype of AML if we classify it by cytogenic abnormalities? How do we characterize it and what is he problem because of the genetic mutation?
Acute promyelocytic leukemia
Translocation of the retinoic acid receptor from chromosome 17 to 15.
Disruption of the RAR blocks maturation of cells so there is a build up of myeloid blasts.
APL is a huge risk for what condition and how do we treat APL?
DIC
ATRA
Let’s say we cant subclassify the AML based on cytogenic abnormalities, so we have to on lineage of immature cells, what are the two High Yield ones?
acute monocytic and acute megakaryoblast
What do the monoblasts in acute monocytic lack and where in the body do they classically infiltrate?
What do the megakaryoblasts lack in acute megakaryoblastic and what high yield association is there?
MPO and infiltrate the gums
MPO and Down syndrome association
Quickly recap and discuss the association acute leukemia has with Down Syndrome?
Down syndrome is highly associated with leukemia. It is associated with ALL in kids over 5 and AML (megakaryoblastic) in kids less than 5.
AML can also arise from an alternative pre existing condition. What is that condition, what are two causes, what are 4 clinical/morphological signs, and how do these patients progress?
Myelodysplasia.
Prior exposure to alkylating agents, like chemo, or radiotherapy
Cytogenic, hypercellular bone marrow, abnormal maturation of cells, and increased blasts (but less than 20%)
Most die from infection or bleeding, but some progress to AML
