Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

IHO Week 4 > Chronic Lymphocytic Leukemia: Clinical Correlates and Issues > Flashcards

Chronic Lymphocytic Leukemia: Clinical Correlates and Issues Flashcards

1
Q

What is the most common leukemia in adults? (EXAM)

A

Chronic lymphocytic leukemia!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the flow cytometry phenotype in CLL? (EXAM)

A

CD5+CD19+CD20+CD23+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the survival, diagnosis and treatment of Chronic Lymphocytic leukemia?

A
  • Median age of diagnosis is 65 years
  • Median survival is 9 years
  • Allogenic stem cell transplant only curative therapy
  • Decision to treat based on age, performance status, symptoms, disease stage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the pathophysiology of CLL?

A
  • CLL is an uncontrolled clonal accumulation of mature lymphocytes
  • Unclear mechanism of initiation
  • Molecular characterization of CLL correlates with clinical outcomes
  • Mutated Ig VH genes –> mutation in this case = better disease prognosis
  • Unmutated Ig VH genes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the key thing you don’t want to miss with CLL? (EXAM)

A

Mantle cell lymphoma!

-mantle cells look similar under the microscope but they’re more nasty to treat than CLL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the difference between CLL and SLL?

A

SLL is the tissue phase of CLL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the phenotype of CLL in flow cytometry? (EXAM)

A

CD5+CD19+CD20+CD23+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the differential for CLL?

A
  • Follicular lymphoma
  • Mantle cell lymphoma with leukemic phase
  • Prolymphocytic leukemia
  • SLVL
  • HCL
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is Mantle cell lymphoma and its phenotype (flow cytometry)?

A

Nasty, aggressive NHL

-CD5+CD23-CyclinD1+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What do you see in a blood smear of CLL?

A
  • Heterogeneity in the appearance of abnormal lymphocytes in CLL
  • See abundance of abnormal lymphocytes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the clinical features of CLL?

A
  • Patient can feel pretty well/normal
  • Often an incidental diagnosis in an symptomatic patient
  • Indolent disease common
  • Progressive adenopathy often correlates with symptoms; fatigue, malaise, weight loss, fevers
  • Progressive bone marrow involvement leads to severe cytopenias (reduction in blood cells), increased risk of infection
  • Autoimmune sequelae and Richter’s transformations are long-term complications
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are some autoimmune complications of CLL?

A
  • AIHA (autoimmune hemolytic anemia)
  • –Coombs’/DAT positive
  • Pure red cell aplasia (bone marrow just not making red cells - look for reticulocytes!)
  • ITP (immune mediated thrombocytopenia)
  • Neutropenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What’s the key red cell aplasia differential diagnosis?

A

Parvovirus B19 - can cause red cell aplasia, most common in adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the two staging systems CLL?

A
  • Rai

- Binet

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the Rai system?

A

Rai 0 - lymphocytosis
Rai 1 - lymphadenopathy
Rai 2 - splenomegaly
Rai 3 - HgB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the Binet system?

A

A: lymphocytosis +/- 1-3 sites lymphadenopathy
B: lymphocytosis with > 3 sites lymphadenopathy
C: lymphocytosis + anemia and/or thrombocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Why is staging important?

A

Higher staging = less median survival

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What does a Rai 0 stage tell you?

A

Median survival: >10 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What does Rai 1 stage tell you?

A

Median survival: 7 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What does Rai 3 stage tell you?

A

Median survival: 2-5 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are poor prognostic factors for CLL?

A
  • Advanced stage at diagnosis
  • Rapid lymphocyte doubling time
  • Diffuse marrow infiltration
  • Advanced age/male gender
  • CLL-PLL (secondary PLL - prolymphocytic leukemia)
  • Abnormal karyotype (abnormal cytogenetics)
  • Elevated Beta2 -microglobulin, soluble CD23
  • CD38+ or ZAP-70+
22
Q

What are CLL-cytogenetic abnormalities, ranked from best to least?

A
  • 13q-
  • Normal karyotype
  • Trisomy 12
  • 11q-
  • 17p-
23
Q

What should you ALWAYS get with CLL? (EXAM)

A

FISH studies (cytogenetics)

24
Q

What do mutated heavy chain (VH) genes indicate in CLL?

A

Better survival

25
Q

What if the lab cannot perform Ig gene mutation (FISH) studies?

A
  • CD38 may be a surrogate marker for Ig gene mutations
  • -Detect via flow cytometry (easily)
  • -CD38+ = unmutated Ig genes = poorer prognosis
  • -CD38- = mutated Ig genes = better prognosis
  • CD38+: 9 year median survival
  • CD38-: >20 years median survival
26
Q

What is ZAP-70 expression? What does it tell us?

A
  • Correlates with Ig VH gene mutations
  • Correlates with CD38+ expression
  • ZAP-70+ = Poorer prognosis
27
Q

What does under 200 CD4+ cells indicate?

A

You have AIDs

28
Q

When should you treat CLL?

A
  • Constitutional symptoms
  • Progressive lymphocytosis
  • Progressive lymphadenopathy
  • Progressive splenomegaly
  • Progressive bone marrow failure
  • Autoimmune complications
  • Richter’s transformation or secondary PLL
  • Your patient is uncomfortable with observation
29
Q

What are these? (don’t memorize)

  • Corticosteroids
  • Alkylating agents
  • Nucleoside analogs (same risk of infection as AIDs)
  • Monoclonal antibodies
  • Combination chemotherapy
  • Allogeneic stem cell transplantation
  • Ibrutinib
A

Therapeutic options for CLL

30
Q

What is the best way to treat CLL?

A
  • Is there an indication to treat??
  • Prognostic factors (stage, CD38 expression, ZAP-70, etc.)
  • Younger, good PS patients
  • –Clinical trail
  • –PCR or FCR
  • –Bendamustine plus rituximab
  • –Ibrutinib
  • Older, more frail patients
  • –Chlorambucil (+/- prednisone)
  • –Bendamustine plus rituximab
  • –Ibrutinib
31
Q

What is the only curative modality for CLL?

A

Allogenic transplant

  • Need matched-sib donor
  • Substantial morbidity/mortality
  • The price of curing CLL is GVHD
32
Q

60 yr, f, excellent health, high WBC:

A

Patient has M0 CLL

33
Q

What does 13q- CLL show on blood smear?

A
  • Normal lymphocytes are dark purple and others are “smudge cells”
  • The mechanical streaking burst the cells - very flimsy
34
Q

What is the key clinical question for leukemias?

A

When was the last time you felt normal?

35
Q

57 yr, M, 3 months fevers, night sweats, speaks with hoarse voice, adenopathy in many areas. What causes hoarse voice and what does blood smear look like?

A
  • Hoarse voice caused by (mediastinal adenopathy) lymph nodes in the chest swelling and paralyzing the recurrent laryngeal nerve (vocal cord)
  • A blood smear shows diffusely infiltrated
  • Treated with PCR immunotherapy
36
Q

What can happen with patients who are in remission from CLL?

A
  • Prolymphocytes seen on blood smear
  • CLL transformed into secondary prolymphocytic leukemia (eccentrically placed nuclei)
  • –Richter’s transformation
  • –New 17p-
  • Poor prognostic sign
  • Demonstrated refractory disease with BM failure and severe cytopenias
37
Q

What does DOE stand for?

A

Dyspenea on exertions

38
Q

65 yr old, F, remission for CLL, has fatigue & DOE, Pale, little tachycardic:

A
  • She has very low hemoglobin
  • Blood smear: very light staining RBCs, also see spherocytes!!
  • Spherocytes = Autoimmune hemolytic anemia! = Diagnosis!!
39
Q

What is orthopnea?

A

Patient gets shot of breath when they’re laying flat - they use many pillows to sit themselves up - sleep in a chair

40
Q

What is tachycardia?

A

Heart rate above 100

41
Q

When do you see spherocytes and nucleated RBCs on blood smears?

A

Presence of autoimmune hemolytic anemia

42
Q

What’s important about AIHA (autoimmune hemolytic anemia)?

A
  • Common auto-immune sequel of CLL
  • Presence of AIHA does NOT necessarily correlate with disease activity
  • Treated successfully with prednisone!
43
Q

Where does erythropoietin come from?

A

Kidneys!

44
Q

59 yr old, M, 10 yrs with CLL, not on therapy, DOE, low hemoglobin, high WBC, Extremely high erythropoietin:

A

Diagnosis?

  • PRCA (pure red cell aplasia - autoimmune phenomenon)
  • Auto-immune neutropenia
  • Immune-mediated thrombocytopenia
45
Q

How long do red cells last in our bodies?

A

About 120 days!

46
Q

What drug has been approved for autoimmune complications of CLL by the FDA recently?

A

Ibrutinib

-For Mantle Cell lymphoma patients who have received one prior therapy

47
Q

What is the therapeutic target for ibrutinib? What is ibrutinib?

A
  • BCR signaling pathway (leads to NF-kB activation)

- BTK inhibitor - orally active (reversible)

48
Q

What is important to know about Imbruvica?

A
  • Less than 1% is really exerted, so there are no renal impairment guidelines
  • Atrial fibrillation is a side effect
  • Extremely expensive = $11,000/month
  • If you stop the drug, it sets up resistance and then you can’t use anything to fight the disease
49
Q

How does Imbruvica (ibrutinib) affect pregnancy?

A
  • Pregnancy class D
  • –Organ malformation and low birth weight in rats
  • –Unclear if it is present in milk or nursing mothers
  • No indication in pediatrics
50
Q

What to know about Ibrutinib?

A
  • Paradigm changing therapy!!

- Oral pill!!

IHO Week 4 (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions