Acute Leukemia

Question 1

2 types of acute leukemia?

Answer 1

Acute Myeloid Leukemias (AML)

Acute Lymphoid Leukemias (ALL)

Question 2

Incidence for AML

Answer 2

1. 7/ 105 individuals < 65 y.o.

15. 9/ 105 individuals > 65 y.o.

Question 3

Acute Myeloid Leukemias Etiology (5)

Answer 3

Antecedent MDS or Rarely MPS
Inherited Predispositions (Down Syndrome)
Chemotherapies: Alkylating Agents, Topoisomerase II Inhibitors, Chloramphenicol, Phenylbutazone
Chemicals & Radiation (Benzene, Pesticides)

Question 4

What is one important characteristic of cancers?

Answer 4

that the Cells of the Cancer Seem to Have Stopped Differentiating at Some Point in their Maturation

Question 5

Classification of AML (FAB)A Morphologic and Histochemical Classification Based on Stage of Maturation Arrest

Answer 5

M0: Minimally Differentiated AML
M1: Myeloblastic Without Differentiation
M2: Myeloblastic With Differentiation
M3: Promyelocytic Acute Leukemia
M4: Myelomonocytic Acute Leukemia
M5: Monocytic Acute Leukemia
M6 Erythroleukemia
M7: Megakaryocytic Acute Leukemia

Question 6

How does WHO classify AML?

Answer 6

More Complicated and More Comprehensive Classification System
This System Incorporates 
Patient History 
Cellular Morphology 
Histochemistry 
Flow Cytometry
Chromosomal Analysis and 
Molecular Evaluation

Question 7

Presenting Symptoms and Signs for AML?

Answer 7

The most common Sxs are Fever, Bleeding, Fatigue and SOB
Stroke-like symptoms or other Thrombotic Complications
Retinal Hemorrhages
Gingival Hyperplasia (Monocytic Leukemias)
Solid Tumors

Question 8

What should immediately raise the possibility of Acute Promyelocytic Leukemia (APL).

Answer 8

stroke like symptoms or other thrombotic complications

Question 9

Lab Findings in AML?

Answer 9

Anemia:  Macrocytic in MDS related cases
WBC:
~35% < 5 x 103 WBC/mm3
~ 45%  5 x 103 – 99 x 103 WBC/ mm3
~ 20% > 100 x 103 / mm3
The Characteristic Peripheral Smear: Blast with a “Leukemic Hiatus”  (Poly and Blasts, but no intermediate forms)
Most Patients will be Thrombocytopenic

Question 10

What do you do If the Patient has Not Entered a Complete Remission (CR)?

Answer 10

Re-treat
Switch to Salvage Regimen such as High Dose Cytarabine
If this Fails Consider Allogeneic HSCT

Question 11

How are new patients treated with AML?

Answer 11

Induction Therapy Consisting of
7 days of Cytarabine given by Continuous IV Infusion
3 Days of an Anthracycline, Usually Daunorubicin.

Question 12

Patient > 70 y.o. Usually Cannot Tolerate Standard Therapy so how do you treat them?

Answer 12

Treatment Usually Consists of Low Dose Decitabine, a DNA Methyltransferase Inhibitor.
Reduced Intensity Conditioning Regimens with Allo-HSCT have also been successful
Intensive Supportive Therapy with Antibiotics, Blood Products, Care of Mucous Membranes

Question 13

Signs and Symptoms of APL

Answer 13

Like Other Patients with Acute Leukemia EXCEPT Bleeding and Thrombosis are Common.
This is Due to Disseminated Intra-Vascular Coagulation (DIC)
DIC is a Syndrome Characterized by Widespread Small Vessel Thrombosis, End Organ Ischemia & Depletion of Clotting Factors

Question 14

What does ATRA do?

Answer 14

ATRA will induce APL Promyelocytes to Differentiate into Mature Myeloid Cells, like Polys etc..
ATRA induces a Complete Remission (CR) is > 90% of Patients

Question 15

What is ATRA used to treat?

Answer 15

APL

Question 16

What is the prognosis of AML?

Answer 16

Prognosis is Better in Young People.

Prognosis Depends on Achieving a CR.

Question 17

What is complete remisson defined as? (3)

Answer 17

< 5% Blasts in the Bone Marrow after Rx
Resolution of Cytogenetic and Molecular Abnormalities in the Bone Marrow
CBC with ANC (Absolute Neutrophil Count) > 103 & Platelets > 105

Question 18

What are 2 general classes of ALL

Answer 18

B Cell & T Cell

disease of children and young adults

Question 19

Etiology and epidemiology of ALL

Answer 19

Trisomy 21, Down Syndrome Associated with  Incidence of ALL
Radiation Exposure Associated with Increased Incidence of T Cell ALL
Many Cases of Unknown Etiology

Question 20

What will the physical exam of someone with ALL reveal?

Answer 20

Commonly Reveal Lymphadenopathy &

Hepato-Splenomegaly

Question 21

ALL’s first peak is in pts less than 20. When is the second peak?

Answer 21

second Peak Incidence of ALL in Individuals > 70 y.o. These Individuals have a Poor Prognosis.

Question 22

What is considered standarded care for patients with ALL?

Answer 22

lumbar puncture is considered Standard in ALL as CNS Involvement is Common and Requires Special RX

Question 23

What is the prognosis of ALL

Answer 23

Long Term Survival in Pediatric ALL > 90%

Long Term Survival in Adult ALL ~ 35%

Question 24

how do you treat ALL?

Answer 24

Vincristine & Prednisone causes Rapid Reduction in ALL Blasts
The Addition of an Anthracycline increases CR to ~ 80%.

Question 25

Epidemiology of all acute leukemia patients?

Answer 25

90 % of Children with ALL will Survive Their Disease
80% of Children with AML will Survive Their Disease
It is Estimated that 1:250 Individuals age 15 – 45 will be a Pediatric Cancer Survivor in the Next Decades

Question 26

What is the 12 step care plan for pediatric patients with leukemia?

Answer 26

Oncology Team Contact Information
Diagnosis, Tests Run, Rx Given and Objective data at the Time of Discharge.
Psychosocial & Nutritional Services Provided
Oncology Team Coordinator of Continuing Care
Likely Course of Recovery
Recommended Periodic Exams & Testing
Possible late and Long Term Health Problems
Signs and Sx of Recurrence or Second Cancers
Possible Effects on Long Term Adjustment and Activites of Daily Living
Potential Legal &/or Financial Problems
Genetic or Family Implications of this Dx
Appropriate Strategies to Prevent Recurrence or Other Illnesses