Chronic Leukemia - Simms

Question 1

Most common leukemia of adults in the western world

Answer 1

Chronic Lymphocytic Leukemia

(70 y/o; M>F)

Question 2

CD5+ on flow cytometry indicates ______

Answer 2

CLL. This is an aberrant marker (belongs to T cell)

(also CD19+/CD20+)

Question 3

CLL is an accumulation of ____ cells

Answer 3

mature (not blastic) neoplastic lymphocytes (mostly B-cells)

Question 4

Answer 4

CLL: “smudge cells” or “basket cells”

(made by making the slide)

Question 5

Bone marrow failure seen in CLL will lead to _____ (3).

Answer 5

1. thrombocytopenia → bleeding
2. anemia
3. neutropenia

Question 6

Answer 6

Bone marrow bx: CLL

Question 7

What does this tell you?

Answer 7

light chain restriction

Question 8

In CLL, del ____ has an intermediate survival, while del ____ has the poorest.

Answer 8

• 11q → DNA repair mutation
• 17p → p53 loss

Question 9

In T-cell CLL, del _____ has extensive lymph node involvement and is aggressive.

Answer 9

11q22 → ATM deletion

Question 10

Unlike B-cell Malignant lymphoma, B-cell CLL manifests as a high expression of _____.

Answer 10

BCL-2 on 18q21 (anti-apoptotic protein→ maintains neoplastic cells)

(this is a different mechanism than some of the lymphomas)

Question 11

In CLL, loss of the tumor suppression is especially prolific in the proliferation centers where tumor cells receive signals for growth and survival through the ______.

Answer 11

Bruton Tyrosine Kinase (BTK)

Question 12

B-cell CLL has mutations in the ______ chain of the VH gene complex

Answer 12

heavy or light

(markers for the VH gene complex are CD38 & ZAP-70)

Question 13

How do we monitor the minimal residual disease (MRD) of leukemias?

Answer 13

cytogenetic screening of marrow or blood

Question 14

Median survival for CLL is ______ w/low tumor burden

Answer 14

4-6 years

Question 15

Lymph node biopsy of CLL will show diffuse effacement by an infiltrate of ______ & proliferation centers (mixed large lymphocytes & loose aggregates)

Answer 15

small lymphocytes

Question 16

Good prognosis: markers for the VH gene complex are CD38 & ZAP-70 are _____ (increased/decreased)

(Immature subtype of CLL)

Answer 16

decreased (means they are less mature)

(Mature sub-type does not have these at all → better prognosis)

Question 17

Answer 17

AML

(below is normal bone marrow)

Question 18

Like AML, CML has CD13+, CD33+, but in opposition to AML, CML is ______ (-).

Answer 18

CD45RA

(also CML has a philadelphia chromosome - short 22)

Question 19

CML lab findings include immature myelocytic cells as well as elevated ______ (2 cell types).

Answer 19

1. basophils
2. eosinophils

Question 20

How can you tell if a myeloblast is mature?

Answer 20

increased granulation in the cytoplasm

Question 21

Answer 21

CML : mixture of mature cells

Question 22

why is uric acid elevated in CML? What can this lead to?

Answer 22

• it is a metabolite of DNA (high cell turnover bc they are dying)
• oligoarticular arthritis (deposition)

Question 23

______ is a unique is a risk factor for CML

Answer 23

Herbicide/pesticide exposure

Question 24

In CML, lab findings include : elevated uric acid, ______(2).

Answer 24

1. anemia
2. high plts

Question 25

Why is there hyperviscosity in CML?

Answer 25

hyperleukocytosis (may manifest as tinnitus, confusion, priapism)

Question 26

Staging of CML: chronic, accelerated and _____ phase.

Answer 26

blast (\>30% blasts in blood or bone marrow) (based on amount of blasts; after blast phase → becomes ALL or AML. usually w/in 5 yrs)

Question 27

CML can transform into ALL, AML or \_\_\_\_\_.

Answer 27

Sweet syndrome

Question 28

1st line of therapy if CML patient is \<45 years old?

Answer 28

bone marrow transplant (imatinib, nilotinib, dasatinib until they can get the transplant)

Question 29

bone marrow biopsy of CML will show macrophages w/\_\_\_\_\_\_.

Answer 29

blue-green coloration ("sea-blue" hisotcytes)

Question 30

In addition to “sea-blue histiocytes”, CML bone marrow bx will show\_\_\_\_\_\_(2)

Answer 30

1. hypercellular marrow: granulocytic & megakaryocytic precursors 2. no fat in marrow

Question 31

Hairy Cell Leukemia has _____ (markers)

Answer 31

1. ***_CD103+_*** 2. Cyclin D1 3. CD11c+ (Lots of B cell markers: CD19, 20, 22, 25. **No CD3, CD5**)

Question 32

\_\_\_\_\_\_ lab finding include: pancytopenia, elevated IL-2, **+ Tartrate-Resistant Acid Phosphatase (aka TRAP)**.

Answer 32

Hairy Cell Leukemia

Question 33

Hairy cell leukemia has a point mutation in \_\_\_\_\_

Answer 33

BRAF gene (downstream of RAS) → proliferation & survival (Ser/Threonine kinase)

Question 34

CBC on Hairy Cell Leukemia

Answer 34

pancytopenia (WBC, anemia, plts)

Question 35

Mycobacterial infection & massive splenomegaly are common features of ______ leukemia.

Answer 35

hairy cell

Question 36

Prognosis for Hairy Cell Leukemia

Answer 36

Excellent (relapse in 5 years possible, but same tx)

Question 37

HTLV-1 can give rise to a progressive \_\_\_\_\_\_\_

Answer 37

demyelinating disease of the CNS

Question 38

Adult T Cell Leukemia/Lymphoma has atypical lymphocytes known as \_\_\_\_\_\_\_

Answer 38

flower cells | (lobulated multinuclei)

Question 39

Adult T Cell Leukemia/Lymphoma has atypical lymphocytes known as \_\_\_\_\_\_\_

Answer 39

"flower" or "clover" T cells (TH) (lobulated multinuclei; also present in Peripheral T-cell Lymphoma)

Question 40

T cell ______ is detectable with Adult T Cell Leukemia/Lymphoma

Answer 40

clonal receptor rearrangement

Question 41

Which diagnostic feature does Adult T cell Leukemia/Lymphoma share with Multiple Myeloma?

Answer 41

Lytic bone lesions | (this leads to hypercalcemia)

Question 42

How would you dx Adult T cell Leukemia/Lymphoma?

Answer 42

PCR or ELISA for HTLV-1 virus

Question 43

What is the most common presentation of HTLV-1?

Answer 43

Adult T cell **leukemia** **(lymphoma is uncommon)**

Question 44

t(9;22) is seen in which diseases?

Answer 44

1. CML 2. AML 3. ALL (don't rely on that alone for diagnosis)

Question 45

Adult T-cell Leukemia/Lymphoma occurs mainly in which 3 geographic locations?

Answer 45

1. Japan 2. W. Africa 3. Caribbean

Question 46

Once Adult T-cell Leukemia/Lymphoma presents symptoms it is aggressive, prognosis?

Answer 46

fatal within months to 1 year despite aggressive chemo