Chronic Leukemia - Simms Flashcards

1
Q

Most common leukemia of adults in the western world

A

Chronic Lymphocytic Leukemia

(70 y/o; M>F)

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2
Q

CD5+ on flow cytometry indicates ______

A

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

(also CD19+/CD20+)

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3
Q

CLL is an accumulation of ____ cells

A

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

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4
Q
A

CLL: “smudge cells” or “basket cells”

(made by making the slide)

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5
Q

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

A
  1. thrombocytopenia → bleeding
  2. anemia
  3. neutropenia
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6
Q
A

Bone marrow bx: CLL

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7
Q

What does this tell you?

A

light chain restriction

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8
Q

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

A
  • 11q → DNA repair mutation
  • 17p → p53 loss
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9
Q

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

A

11q22 → ATM deletion

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10
Q

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

A

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

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

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11
Q

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 ______.

A

Bruton Tyrosine Kinase (BTK)

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12
Q

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

A

heavy or light

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

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13
Q

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

A

cytogenetic screening of marrow or blood

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14
Q

Median survival for CLL is ______ w/low tumor burden

A

4-6 years

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15
Q

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

A

small lymphocytes

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16
Q

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

(Immature subtype of CLL)

A

decreased (means they are less mature)

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

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17
Q
A

AML

(below is normal bone marrow)

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18
Q

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

A

CD45RA

(also CML has a philadelphia chromosome - short 22)

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19
Q

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

A
  1. basophils
  2. eosinophils
20
Q

How can you tell if a myeloblast is mature?

A

increased granulation in the cytoplasm

21
Q
A

CML : mixture of mature cells

22
Q

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

A
  • it is a metabolite of DNA (high cell turnover bc they are dying)
  • oligoarticular arthritis (deposition)
23
Q

______ is a unique is a risk factor for CML

A

Herbicide/pesticide exposure

24
Q

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

A
  1. anemia
  2. high plts
25
Q

Why is there hyperviscosity in CML?

A

hyperleukocytosis

(may manifest as tinnitus, confusion, priapism)

26
Q

Staging of CML: chronic, accelerated and _____ phase.

A

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)

27
Q

CML can transform into ALL, AML or _____.

A

Sweet syndrome

28
Q

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

A

bone marrow transplant

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

29
Q

bone marrow biopsy of CML will show macrophages w/______.

A

blue-green coloration (“sea-blue” hisotcytes)

30
Q

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

A
  1. hypercellular marrow: granulocytic & megakaryocytic precursors
  2. no fat in marrow
31
Q

Hairy Cell Leukemia has _____ (markers)

A
  1. CD103+
  2. Cyclin D1
  3. CD11c+

(Lots of B cell markers: CD19, 20, 22, 25. No CD3, CD5)

32
Q

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

A

Hairy Cell Leukemia

33
Q

Hairy cell leukemia has a point mutation in _____

A

BRAF gene (downstream of RAS) → proliferation & survival

(Ser/Threonine kinase)

34
Q

CBC on Hairy Cell Leukemia

A

pancytopenia (WBC, anemia, plts)

35
Q

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

A

hairy cell

36
Q

Prognosis for Hairy Cell Leukemia

A

Excellent

(relapse in 5 years possible, but same tx)

37
Q

HTLV-1 can give rise to a progressive _______

A

demyelinating disease of the CNS

38
Q

Adult T Cell Leukemia/Lymphoma has atypical lymphocytes known as _______

A

flower cells

(lobulated multinuclei)

39
Q

Adult T Cell Leukemia/Lymphoma has atypical lymphocytes known as _______

A

“flower” or “clover” T cells (TH)

(lobulated multinuclei; also present in Peripheral T-cell Lymphoma)

40
Q

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

A

clonal receptor rearrangement

41
Q

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

A

Lytic bone lesions

(this leads to hypercalcemia)

42
Q

How would you dx Adult T cell Leukemia/Lymphoma?

A

PCR or ELISA for HTLV-1 virus

43
Q

What is the most common presentation of HTLV-1?

A

Adult T cell leukemia

(lymphoma is uncommon)

44
Q

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

A
  1. CML
  2. AML
  3. ALL

(don’t rely on that alone for diagnosis)

45
Q

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

A
  1. Japan
  2. W. Africa
  3. Caribbean
46
Q

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

A

fatal within months to 1 year despite aggressive chemo