394 - 395 - Leukemias

Question 1

What populations are most often affected by ALL?

Answer 1

Age < 15 years, Down syndrome patients

Question 2

How does T-cell ALL present?

Answer 2

mediastinal mass (leukemic infiltration of the thymus)

Question 3

What are the blood smear and bone marrow findings in ALL?

Answer 3

↑ lymphoblasts

Question 4

What serum markers are present in ALL?

Answer 4

1. TdT+ (marker of pre-T and pre-B cells)

2. CD10+ (pre-B cells only)

Question 5

How responsive is ALL to therapy?

Answer 5

It is the most responsive leukemia to therapy

Question 6

What are some places especially at risk for ALL spread?

Answer 6

CNS and testes

Question 7

What translocation in ALL is associated with a better prognosis?

Answer 7

t(12, 21)

Question 8

What lymphoma is considered the counterpart to CLL?

Answer 8

small lymphocytic lymphoma (SLL)

Question 9

What is the difference b/w SLL and CLL?

Answer 9

CLL has ↑ peripheral blood lymphocytosis or bone marrow involvement

Question 10

What is the normal age range for CLL patients?

Answer 10

> 60 years

Question 11

What markers indicate CLL?

Answer 11

CD20+, CD5+

Question 12

IS CLL of T or B cell origin?

Answer 12

B cell

Question 13

What is the presentation and course of CLL?

Answer 13

Often asymptomatic, progresses slowly

Question 14

What is found in the peripheral blood smear in CLL?

Answer 14

smudge cells

Question 15

What condition involving RBCs is associated with CLL?

Answer 15

autoimmune hemolytic anemia

Question 16

Who is affected by hairy cell leukemia?

Answer 16

Adults/elderly

Question 17

What type of cells make up hairy cell leukemia?

Answer 17

Mature B-cells with filamentous, hair-like projections

Question 18

How would you diagnose hairy cell leukemia?

Answer 18

stain for TRAP (becoming outdated) or do flow cytometry

Question 19

What would you find on bone marrow aspiration in hairy cell leukemia?

Answer 19

It would be a dry tap because of marrow fibrosis

Question 20

How do you treat Hairy cell leukemia?

Answer 20

Cladribine (2-CDA) - an adenosine analog (inhibits adenosine deaminase)

Question 21

Who gets AML?

Answer 21

older people (median onset 65 years)

Question 22

What is seen on blood smear in AML?

Answer 22

1. auer rods
2. peroxidase+ cytoplasmic inclusions (mostly in M3 subtype)
3. ↑↑↑ circulating myeloblasts

Question 23

What are the risk factors for AML?

Answer 23

1. alkylating chemotherapy
2. radiation
3. myeloproliferative disorders
4. Down syndrome

Question 24

What is another name for the M3 subtype of AML?

Answer 24

Acute Promyelocytic Leukemia (APL)

Question 25

What translocation leads to the M3 subtype of AML (APL)?

Answer 25

t(15;17)

Question 26

How do you treat APL?

Answer 26

all-trans retinoic acid (vitamin A) → induces differentiation of myeloblasts

Question 27

What is a hematologic complication of M3 AML and its treatment?

Answer 27

DIC is a common presentation and can be induced by chemotherapy due to release of Auer rods

Question 28

Who gets CML?

Answer 28

peak incidence 45-85 years -- median age at diagnosis 64 years

Question 29

What is the translocation in CML?

Answer 29

t(9;22) - Philadelphia chromosome

Question 30

What is on the Philadelphia chromosome?

Answer 30

bcr-abl

Question 31

What cells make up CML?

Answer 31

myeloid stem cells: ↑ neutrophils, metamyelocytes, basophils

Question 32

What might you find on physical exam of the abdomen in CML?

Answer 32

splenomegaly

Question 33

What is the prognosis of CML?

Answer 33

It responds to imatinib; can accelerate and transform to AML or ALL (blast crisis)

Question 34

What is imatinib?

Answer 34

a small-molecule inhibitor of bcr-abl tyrosine kinase

Question 35

What is the trade name of imatinib?

Answer 35

Gleevec