PHR 957: Block 2 Midterm Exam
With Correct Solution
What is the most prevalent adult leukemia? - ANSWER Chronic Lymphocytic Leukemia
(CLL)
T or F: CLL is predominantly in peripheral circulation - ANSWER True
What type of cancer is CLL? - ANSWER B cell cancer
*affects lymphocytes - accumulate in blood, bone marrow, lymph nodes, liver and
spleen*
T or F: Individuals who present with CLL generally show no symptoms - ANSWER True;
usually takes ~3 years to start seeing symptoms
Signs/Symptoms of CLL (6) - ANSWER 1. Fatigue
2. Weight loss
3. Early satiety (splenomegaly)
4. Petechiae (thrombocytopenia)
5. Palpable lymph nodes (lymphadenopathy)
6. Recurrent infections or bleeding
How is CLL diagnosed? - ANSWER *Blood sample*
> 5x10^9 monoclonal B-lymphocytes in peripheral blood
Flow cytometry
FISH
What does the flow cytometry show in patients with CLL? - ANSWER CD5 +
CD10 -
CD19 +
CD20 dim
surface immunoglobulin dim
,CD23 +
cyclin D1 -
What does the FISH show in patients with CLL? - ANSWER 11q-
13q-
trisomy 12
17p-
What 2 staging systems are used for CLL? - ANSWER *1. Rai*
2. Binet
What is Rai stage 0 CLL? - ANSWER > 5x10^9 clonal B cells and > 40% lymphocytes in
bone marrow
What is Rai stage I CLL? - ANSWER Stage 0 with enlarged nodes on physical exam
What is Rai stage II CLL? - ANSWER Stage 0-1 with splenomegaly, hepatomegaly OR
both
What is Rai stage III CLL? - ANSWER Stage 0-II with hemoglobin <100g/dL or hematocrit
<33%
What is Rai stage IV CLL? - ANSWER Stage o-III with platelets <100,000 mcL
What are prognostic factors of CLL? (2) - ANSWER 1. Mutated IGH (immunoglobulin
heavy chain mutation)
2. 13q- only
What are unfavorable prognostic factors of CLL? (6) - ANSWER 1. Non-mutated IGHV
2. 11q-
3. 17p-
4. TP53 mutation
5. Elevated serum B2 microglobulin
6. Higher state
T or F: A patient was diagnosed with CLL rai stage II after experiencing symptoms such
as weight loss and fatigue. After labs pt shows no signs of end organ dysfunction of
anemia/thrombocytopenia. She would not receive treatment because she has not
advanced to stage III or IV. - ANSWER False; any symptoms are an indication for
treatment
, What are indications for treatment of CLL? (5) - ANSWER 1. Advanced stage (rai III-IV or
binet C)
2. Symptoms (fatigue, weight loss, night sweats, fever w/o infection)
3. End organ dysfunction
4. Progressive bulky disease (enlarged spleen or lymph nodes)
5. Progressive anemia or thrombocytopenia
What treatment is used for young and healthy patients diagnosed with CLL without
del(17p) or TP53 mutation? (3) - ANSWER 1. Ibrutinib
2. Acalabrutinib +/- Obinutuzumab
3. Venetoclax + Obinutuzumab
What treatment is used for old/frail patients diagnosed with CLL without del(17p) or
TP53 mutation? - ANSWER Same as healthy/young pts
What treatment is used for patients diagnosed with CLL with del(17p) or TP53 mutation?
(3) - ANSWER 1. Acalabrutinib +/- Obinutuzumab
2. Ibrutinib
3. Venetoclax + Obinutuzumab
Ibrutinib MOA - ANSWER Potent and irreversible inhibitor of Bruton's tyrosine kinase
(BTK)
Inhibiting BTK results in decreased malignant B-cell proliferation and survival
Acalabrutinib MOA - ANSWER Selective and irreversible second-generation Bruton's
tyrosine kinase (BTK) inhibitor
How do Ibrutinib's and Acalabrutinib's MOA differ? - ANSWER A is more selective for
BTK and less toxic
How is Ibrutinib administered? - ANSWER 420mg PO w/ full glass of water
Ibrutinib: ADEs (8) - ANSWER 1. Thrombocytopenia
2. Neutropenia
3. Anemia
4. Diarrhea
5. Fatigue
6. Musculoskeletal pain
With Correct Solution
What is the most prevalent adult leukemia? - ANSWER Chronic Lymphocytic Leukemia
(CLL)
T or F: CLL is predominantly in peripheral circulation - ANSWER True
What type of cancer is CLL? - ANSWER B cell cancer
*affects lymphocytes - accumulate in blood, bone marrow, lymph nodes, liver and
spleen*
T or F: Individuals who present with CLL generally show no symptoms - ANSWER True;
usually takes ~3 years to start seeing symptoms
Signs/Symptoms of CLL (6) - ANSWER 1. Fatigue
2. Weight loss
3. Early satiety (splenomegaly)
4. Petechiae (thrombocytopenia)
5. Palpable lymph nodes (lymphadenopathy)
6. Recurrent infections or bleeding
How is CLL diagnosed? - ANSWER *Blood sample*
> 5x10^9 monoclonal B-lymphocytes in peripheral blood
Flow cytometry
FISH
What does the flow cytometry show in patients with CLL? - ANSWER CD5 +
CD10 -
CD19 +
CD20 dim
surface immunoglobulin dim
,CD23 +
cyclin D1 -
What does the FISH show in patients with CLL? - ANSWER 11q-
13q-
trisomy 12
17p-
What 2 staging systems are used for CLL? - ANSWER *1. Rai*
2. Binet
What is Rai stage 0 CLL? - ANSWER > 5x10^9 clonal B cells and > 40% lymphocytes in
bone marrow
What is Rai stage I CLL? - ANSWER Stage 0 with enlarged nodes on physical exam
What is Rai stage II CLL? - ANSWER Stage 0-1 with splenomegaly, hepatomegaly OR
both
What is Rai stage III CLL? - ANSWER Stage 0-II with hemoglobin <100g/dL or hematocrit
<33%
What is Rai stage IV CLL? - ANSWER Stage o-III with platelets <100,000 mcL
What are prognostic factors of CLL? (2) - ANSWER 1. Mutated IGH (immunoglobulin
heavy chain mutation)
2. 13q- only
What are unfavorable prognostic factors of CLL? (6) - ANSWER 1. Non-mutated IGHV
2. 11q-
3. 17p-
4. TP53 mutation
5. Elevated serum B2 microglobulin
6. Higher state
T or F: A patient was diagnosed with CLL rai stage II after experiencing symptoms such
as weight loss and fatigue. After labs pt shows no signs of end organ dysfunction of
anemia/thrombocytopenia. She would not receive treatment because she has not
advanced to stage III or IV. - ANSWER False; any symptoms are an indication for
treatment
, What are indications for treatment of CLL? (5) - ANSWER 1. Advanced stage (rai III-IV or
binet C)
2. Symptoms (fatigue, weight loss, night sweats, fever w/o infection)
3. End organ dysfunction
4. Progressive bulky disease (enlarged spleen or lymph nodes)
5. Progressive anemia or thrombocytopenia
What treatment is used for young and healthy patients diagnosed with CLL without
del(17p) or TP53 mutation? (3) - ANSWER 1. Ibrutinib
2. Acalabrutinib +/- Obinutuzumab
3. Venetoclax + Obinutuzumab
What treatment is used for old/frail patients diagnosed with CLL without del(17p) or
TP53 mutation? - ANSWER Same as healthy/young pts
What treatment is used for patients diagnosed with CLL with del(17p) or TP53 mutation?
(3) - ANSWER 1. Acalabrutinib +/- Obinutuzumab
2. Ibrutinib
3. Venetoclax + Obinutuzumab
Ibrutinib MOA - ANSWER Potent and irreversible inhibitor of Bruton's tyrosine kinase
(BTK)
Inhibiting BTK results in decreased malignant B-cell proliferation and survival
Acalabrutinib MOA - ANSWER Selective and irreversible second-generation Bruton's
tyrosine kinase (BTK) inhibitor
How do Ibrutinib's and Acalabrutinib's MOA differ? - ANSWER A is more selective for
BTK and less toxic
How is Ibrutinib administered? - ANSWER 420mg PO w/ full glass of water
Ibrutinib: ADEs (8) - ANSWER 1. Thrombocytopenia
2. Neutropenia
3. Anemia
4. Diarrhea
5. Fatigue
6. Musculoskeletal pain
