Acute Leukemias Flashcards Preview

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Flashcards in Acute Leukemias Deck (25)
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1

List risk factors for acute leukemia

Previous chemotherapy (DNA alkylating Agents and Topoisomerase inhibitors)

Ionizing radiation

Tobacco Smoke

Benzene Exposure

Genetic syndromes; downs, bloom, fanconi anemia, ataxia telangiectasia

2

List common signs and symptoms exhibited by patients with acute leukemia at initial presentation, and explain the reasons for these findings.

Fatigue, malaise, pallor, dypsnea

Bruising, petechiae, hemorrhage

Fever, infections

Rare:
Thrombotic events due to blood viscosity
DIC, initiated by leukemic cells in AML
Direct infiltration of skin/gums/nodes/other tissue

3

ALL- Acute Lymphoblastic Leukemia

Neoplasm of precursor lymphoid

B-ALL or T-ALL

incidence of 1-5/100,000

75% of ALL occur in children less than 6 years old

4

How to diagnose ALL

Blast is majority of marrow cells (Packed Marrow)

Peripheral blood WBC count increased/mild increase/normal/decreased

5

Cell Markers for ALL

Immaturity markers- CD34
Lymphoblast marker- TdT (enzyme)
Markers of B Cell- CD19N CD22
Markers of T Cells- CD3, CD7

6

B-ALL

80-85% of cases of ALL

B-lymphoblasts lack mature b cell markers

Typical ALL of childhood

7

T(9;22) of B-ALL

t(9;22), deriavtive of Philadelphia Chromosome, 25% of adult ALL, but only 2% of children ALL

Different breakpoint than CML

Unfavorable prognosis

8

11p23 MLL of B-ALL

Poor prognosis
MLL may be rearranged
Seen in neonates and young infants

9

t(12;21) ETV6-RUNX1` of B-ALL

25% of child B-ALL
Favorable Prognosis

10

T-ALL

Minority of ALL, 25%
More frequently in adolescents and young adults
Males over females
Elevated WBC count
Often presents with mediastinal mass

11

ALL Treatment

Good prognosis disease for the most part in chidlren
Remission rates of >90%, cure rate ~80%

For adults, pronosis is worse

12

Prognosis factors for ALL

Age (Kids do better, older gets worse)
# of WBC count
If it's T or B lymphoblast (T is worse)
Hyperdiploidy > Hypodiploidy
Speed of response to therapy (slow is bad)

13

AML, Acute Myeloid Leukemia

More heterogenous than ALL, so more types

Adult disease mostly, average age is 65 for diagnosis

3/100,000 per year, similar to ALL

14

Diagnosing AML

presence of >=20% myeloblasts in marrow or blood

Look for markers: CD34 (immaturity), CD117/Myeloperoxidase (myeloid markers)

15

Auer Rods

Fused granules forming small stick like structures in cytoplasm. PResence = blast of myeloblast, and abnormal

16

AML with t:8;21); RUNX1-RUNX1T1

5%
Younger patients
AML with maturation
Mutation of a core binding factor needed for differentiation
Relatively good prognosis
Presence = diagnosis

17

AML with inv(16) or t(16;16); CBFB-MYH11

5-10% of AML
Younger patients
Good prognosis
Presence = diagnosis
Smear has presence of Beso Eos

18

APL with t(15;17), PML-RARA

5-10%
acute promyelotic leukemia, accuulation of promyelocytes
Hypergranular morphology
Multiple auer rods
Presence = diagnosis

19

Why is t(15;17) PML RARA so important

1. RARA gene = retinoic acid receptor, required for differentiation

Fusion protein encodes for defective receptor, so poor signalling. Works are higher doses of ligand

Can treat patients with ATRA, which is high dose retinoic acid

2. APL is associated with DIC, so best to avoid that as early as possible

20

AML with t(1;22) RMB15-MKL1

Megakaryoblastic differentiation
Common in infants with downs syndrom
Relatively good prognosis

21

AML with 11q23; MLL gene

Poor prognosis, monocytic differentiation

22

Therapy Related AML (T-AML)

Alkylating agent

2-5year latency, complex karyotype, usually progress to AML through MDS

Poor prognosis

23

Therapy Related AML (T-AML)

Topoisomerase Inhibition

1-2 year latency, just reaarangement of 11q23, does not progress to AML

Poor prognosis

24

Molecular Markets to predict prognosis

FLT 3, most important, poor prognosis, trumps the other two

Nucleophosphin1 (NPM1), good prognosis

CEBPA, good prognosis

25

Leukemic Stem Cell

Cell with potential for self renewel for all different types of myeloid cells (granulocytic, monocytic, erythroid, megakaryocytic).

An inexhaustable supply