Adult Leukemias

Question 1

When you have an abnormality i the blood cells that causes either uncontrollable proliferation, or stops cell regulated cell death, it is called…

Answer 1

Leukemia. Characterized by growth (Acute=fast, Chronic=slow) and type of cell affected.

Question 2

What is not normal on a peripheral smear?

Answer 2

Blasts- REFER!

Question 3

Erythrocytes, megakaryocytes, monocytes, and granulocytes come from what stem cell lineage?

Answer 3

Myeloid. T lymphocyte, NK Lymphocyte, and B lymphocyte come from lymphoid stem cell.

Question 4

Most common pediatric cancer

Answer 4

Acute lymphocytic leukemia (ALL)

Question 5

T/F: Most Adults with ALL are cured while children relapse?

Answer 5

False: Switch

Question 6

Presenting signs/symptoms of ALL

Answer 6

• Bone marrow failure- Anemiam Thrombocytopenia, neutropenia

- Leukemic infiltration- Bone pain, HA, organomegaly, neurologic symptoms, lymphadenopathy

Question 7

Diagnostics for ALL

Answer 7

• CBC with manual diff
• Tumor lysis labs
• Coags
• CXR/CT
• Bone marrow aspirate
• Spinal tap

Question 8

ALL is characterized by the uncontrolled proliferation of ________. This limits the production of other cells by overcrowding and inhibits cell growth and differentiation.

Answer 8

Lymphoblasts

Question 9

Most common type of cell in ALL

Answer 9

Precursor B-Cell

Question 10

Burkit-cell leukemia and frequently occurs in HIV patients involves what cell line

Answer 10

Mature B cell

Question 11

A poor prognosis and associated with HTLV-1 infection. WHat type of cell in ALL?

Answer 11

T-cell

Question 12

3 phases in ALL treatment

Answer 12

1) Induction- includes vincristine, CCS, anthracycline
2) Post-remission consolidation- rotational consolodation, stem cell transplant
3) Maintenance therapy: Includes Methotrexate, 6-mercaptopurine

Question 13

ALL: Prognostic factors

Answer 13

• Age
• Leukocyte count at presentation
• Time to achieve CR
• Immunophenotype
• Cytogenic abnormalities

Question 14

A High WBC at presentation in AML means what?

Answer 14

Poor prognostic factor- WBC100k association with induction death

Question 15

Treatment of choice for ALL

Answer 15

Bone marrow transplant

Question 16

T/F: The incidence of AML is 5X greater than ALL

Answer 16

True

Question 17

Describe the clinical presentation of AML

Answer 17

Abrupt onset (1-6 month prodromal period)

• Similar symptoms to ALL- fatigue, flulike, bleeding, petechiae, purpura, gingival bleeding, GI bleeding, urinary tract bleeding due to decreased platelets.
• Increased susceptibility to infections due to neutropenia
• Enlarged spleen

Question 18

Detection and Dx for AML

Answer 18

• Blood counts, abnormal blood counts lead to detection of AML: Thrombocytopenia, anemia, increased leukocytes.
• Chromosomal abnormalities
• Presence of Auer rods (structres found in myeloblasts, myelocytes, and monoblasts)
• Bone marrow aspiration biopsy for definitive diagnosis.

Question 19

True/False: If 30% blast cells are present, acute leukemia is confirmed?

Answer 19

True- DDX is made from staining procedure. Immunophenotyping as in ALL establishes diagnosis in 90% of cases.

Question 20

Pathology of AML

Answer 20

• Proliferation of precursor cells that have lost the ability to differentiate.
• It involved the hematopoietic stem cells or pluripotent cells
• Results in the gradual accumulation of undifferentiated cells in marrow or other organs.

Question 21

French-American-British Classification of AML

Answer 21

M0- Undifferentiated (MPO negative)
M1- Without maturation
M2- With granulocytic maturation
M3- Acute promyelocytic
M4- Myelomonocytic
M5- Monocytic
M6- Erythroleukemia
M7- Magakaryoblastic

Question 22

Treatment of AML

Answer 22

• Intensive Chemo: Induction, consolidation

- Stem cell transplant

Question 23

A distinct clinical and biologic subtype of AML.

• The most curable AML.
• Separate treatment algorithim from other AML subtypes
• T (15:17) results in fusion transcription gene (PML-RAR a)

WHAT IS IT?

Answer 23

Acute Promyelocytic leukemia (APL)

Question 24

T/F: AML M3 is initially treated differently?

Answer 24

True

Question 25

T/F: Cytogenetics provides critical prognostic info but it is not used to dictate treatment.

Answer 25

False- It now dictates treatment

Question 26

T/F: Chronic Leukemias is the overproduction of blood cells, may not need immediate treatment, and is sometimes found on routine health care maintenance?

Answer 26

True

Question 27

What is the most common leukemia?

Answer 27

``` CLL -Accounts for 30% of leukemias -CLL 2X as common as CML -Incidence increases with age, rare under 35 Males more common. ```

Question 28

Clinical presentation of CLL

Answer 28

- Usually asymptomatic - Bruising (thrombocytopenia) - Early satiety (splenomegaly (late)) - Recurrent infections - Lymphadenopathy

Question 29

T/F: Male sex, White ancestry and Family hx are risk factors for CLL

Answer 29

True

Question 30

CLL Lab Findings:

Answer 30

- Lymphocytosis: NO blasts!! All mature lymphocytes. - Hypogammaglobinemia - Smudge cells

Question 31

CLL Work Up includes

Answer 31

- Adequate Hx/physical - CBC, Differential, PLT count - CMET, LDH, B2-microglobulin - Occasionally: Direct coombs, bone marrow aspirate/biopsy, serum immunoglobulins - Retic count - CXR/abdominal/pelvic - Flow cytometry - Cytogenetics - Molecular studies-Ig receptor gene rearrangements

Question 32

CLL Staging: Rai Staging System

Answer 32

- Low (0)= Lymphocytosis in PB and BM - Intermediate (I-II)= Lymphocytosis, lymphadenopathy, hepatosplenomegaly - High (III-IV)= Lymphocytosis, anemia, thrombocytopenia

Question 33

CLL Staging: Binet Staging

Answer 33

``` A= HGB >10, PLT>100K, 10, PLT>100k, 3 or more nodal areas involved C= HGB ,10, PLT<100K, or both, independent of nodal ares involved ```

Question 34

T/F: 1) CLL is traditionally an illness of younger individuals? 2) CLL well patients do not require therapy? 3) IVIG monthly replacement if hx of bacterial infections (management not treatment)? 4) Earlier/ more aggressive conventional therapy has shown to improve survival or cure of patients?

Answer 34

1) False- older pts 2) True 3) True 4) False: not clearly known to improve

Question 35

Initial therapies in treatment of CLL include:

Answer 35

- Oral chemo - Single agent IV chemo - Combo therapy - Campath-monoclonal antibody

Question 36

What is this: Accounts for 20-30% of all leukemias. Rare in children. Uncommon before age 21. Peaks in 40s. Males slightly more common.

Answer 36

CML

Question 37

The etiology of CML is unknown. Maybe linked to radiation, benzene? What chrmosome is present in 95% of CML patients.

Answer 37

Philadelphia chromosome. Abnormal Ch 22- loss of part of long arm.

Question 38

What are prognostic indicators dependent on in CML?

Answer 38

- Spleen size - PLT count - Hematocrit (% of erythrocytes in blood volume) - Gender - % of blood myeloblasts (immature BM cells)

Question 39

What phase of CML is this: Median survival of 3.5-5 years before transformation. Asymptomatic in 15-40%. May have abdominal complaints/exam consistent with organomegaly.

Answer 39

Chronic phase

Question 40

What phase of CML is this: Median survival 18 months. Peripheral blasts >15%. Increased basophils. Difficult to control white count. Thromocytopenia. Patient with more symptoms such as fever, night sweats, and wt. loss.

Answer 40

Accelerated phase

Question 41

What phase of CML is this? Equivalent to AML. Median survival 3-6 months. Signs include peripheral blasts >30%, marrow blasts >30%, clumps of blasts on BM bx, extramedullary chloromas. Symptoms are similar to accelerated phase.

Answer 41

Blast crisis

Question 42

Lab findings in CML include:

Answer 42

- Leukocytosis | - Thrombocytosis (Sometimes over 1mil.)

Question 43

CML Diagnostic test:

Answer 43

Peripheral blood or bone marrow | -Philadelphia chromosome- translocation (9;22). Involves bcr/abl. Tested by FISH cytogenetics

Question 44

How would you treat stable CML?

Answer 44

Gleevec- tyrosine kinase inhibitor

Question 45

How would you treat unstable CML?

Answer 45

- Admission to control counts- Leukapheresis, hydrea, blast crisis treat as AML. - Medication failures: Stem cell transplant

Question 46

What is this: A malignant tumor of myeloid cells. Can be found in kidney, skin, brain, bowel, etc. Solid tumor may be only sight of disease (bone marrow clear). Still treat systemically.

Answer 46

Granulocytic Sarcoma

Question 47

The take home message with acute leukemia is to not mess around. This means you should...

Answer 47

REFER!