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MCC > Wk 4 Lymphoid and Leukemia > Flashcards

Wk 4 Lymphoid and Leukemia Flashcards

1
Q

What are categorized as lymphoid neoplasms?

A
  1. lymphomas
  2. lymphocytic leukemias
  3. plasma cell neoplasms
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2
Q

What type of cell dominates in lymphoid neoplasms?

A

B-cells (~90%)
T-cells (KN/T-cell) (~10%)

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3
Q

What are 4 characteristics of leukemia?

A
  1. clonal expansion of hematopoietic cells
  2. predominantly in blood and/or BM
  3. can be lymphoid or myeloid
  4. can be immature (acute) or mature (chronic)
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4
Q

What are 4 characteristics of lymphoma?

A
  1. clonal expansion of hematpoietic cells
  2. predominantly in lymph nodes or other tissues
  3. lymphoid lineage
  4. cells are MATURE
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5
Q

What are 5 categories of lymphoid neoplasms?

A

1.
Precursor B-cell neoplasms
Immature B-cell (lymphoblast)
2.
Peripheral B-cell neoplasms
Mature B-cell
3.
Precursor T-cell neoplasms
Immature T-cell (lymphoblast)
4.
Peripheral T-cell and NK-cell neoplasms
Mature T- or NK-cell
5.
Hodgkin lymphoma

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6
Q

What are 5 categories of lymphoid neoplasms?

A

1.
Precursor B-cell neoplasms
Immature B-cell (lymphoblast)
2.
Peripheral B-cell neoplasms
Mature B-cell
3.
Precursor T-cell neoplasms
Immature T-cell (lymphoblast)
4.
Peripheral T-cell and NK-cell neoplasms
Mature T- or NK-cell
5.
Hodgkin lymphoma

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7
Q

What does clonal expansion occur secondary to?

A
  1. antigen exposure
  2. chromosomal rearrangements
  3. genetic mutations
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8
Q

What technique is used to determine whether B cells are clonal?

A

flow cytometry or PCR can measure unique immunoglobulins

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9
Q

How are B-cell leukemias/lymphomas classified?

A

By cell of origin

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10
Q

Know this

A
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11
Q

What CD antigens do I need to know?

A
  1. CD3 – T-cell lineage marker
  2. CD19 – B-cell lineage marker
  3. CD20 – mature B cell marker
  4. CD34 – immaturity marker
  5. Kappa & Lambda light chains – assessment of B-cell clonality
  6. MPO – myeloid lineage marker
  7. TdT – immature lymphocyte marker
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12
Q

How are lymphomas classified?

A
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13
Q

What are the stages of lymphoma?

A

Stage I – one lymph node region or single non-lymphoid organ
Stage II – two or more lymph node regions on same side of diaphragm
Stage III – two or more lymph node regions above and below diaphragm, may include spleen
Stage IV – widespread involvement of non- lymphoid organs and/or bone marrow

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14
Q

What are 6 B-cell lymphomas?

A
  • Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)
  • Mantle cell lymphoma
  • Follicular lymphoma
  • Diffuse large B-cell lymphoma
  • Burkitt lymphoma
  • Hodgkin lymphoma
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15
Q

CLL

A
  1. ~70 yo
  2. Usually presents in leukemic phase (CLL in lymph nodes is SLL) and asymptomatic
  3. increased WBC w/ absolute lymhocytosis
  4. blood smear - small mature lymphocytes, clumped chromatin, scant cytoplasm
  5. Tx - Rituximab (anti-CD20) or Ibrutinib (kinase inhibitor) often only when WBC rapidly increase or evidence of disease progression or symptomatic
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16
Q

Follicular lymphoma

A
  1. common, 50-60 yo, Caucasians
  2. dx w/ lymphadenopathy
  3. chromosomal translocation t(14;18) IGH-BCL2 (-> overexpression of BCL2 (anti-apoptotic protein)
  4. Tx:
    - low-grade (good px) - radiation or Rituximab
    - high-grade (worse px) - R-CHOP
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17
Q

Mantle cell lymphoma

A
  1. 60 yo
  2. dx w/ lymphadenopathy & hepatosplenomegaly
  3. monomorphic small lymphocytes in lymph nodes
  4. chromosomal translocation t(11;14) CCND1-IGH (-> overexpression of Cyclin D1)
  5. aggressive, incurable Tx w/ chemotherapy then stem cell transplant
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18
Q

Diffuse Large B-cell Lymphoma

A
  1. 70 yo
  2. rapidly enlarging tumor mass at single or multiple sites (lymph nodes or other tissues)
  3. B symptoms (fever, night sweats, weight loss) may be present
  4. lymph node architecture diffusely effaced by large atypical cells
  5. can have translocation w/ MYC, BCL2 or BCL6 gene loci
  6. worse px
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19
Q

Burkitt Lymphoma

A
  1. Common in peds
  2. 3 forms: endemic, sporadic, immunodeficiency-associated
  3. lymph node w/ “starry sky” appearance
  4. chromosomal translocation t(8;14) MYC-IGH -> overexpression of MYC oncogene
  5. tx chemotherapy (diff regimens for adults and peds)
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20
Q

What are the 3 forms of Burkitt Lymphoma?

A
  1. Endemic - equatorial Africa, children, jaw mass, EBV+
  2. Sporadic - worldwide, peds, young adults, elderly, abdominal mass, EBV-
  3. immunodeficiency-associated - HIV+ patients, LM involvement
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21
Q

Hodgkin Lymphoma

A
  1. bimodal age dist: young adults and elderly
  2. bulky lymphadenopathy and/or mediastinal mass (B symptoms common)
  3. Lymph node w/ large atypical cells w/ eosinophilic nuclei (Hodgkin cells) or large atypical binucleate cells (Reed-Sternberg cells)
  4. Tx chemotherapy +/- localized radiation therapy
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22
Q

Lymphoma comparison chart

A
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23
Q

What is leukemia?

A

A hematologic neoplasm
-clonal expansion of immature cells (lymphoid or myeloid)
-clone resides in the BM
-malignant cells spill out to peripheral blood

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24
Q

What are the terms when clone of hematologic neoplasm is found outside the BM?

A
  1. lymphoma - when clone is lymphoid
  2. myeloid sarcoma - when clone is myeloid
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25
Q

Subdivisions of leukemia

A
  1. acute - neoplastic cells are immature precurors
  2. chronic - comprised of mature lymphoid/myeloid cells
26
Q

Lymphoma classification

A
27
Q

What are most non-Hodgkin lymphomas?

A

B-cell makes up 80%
-can be indolent or aggressive/high-grade

28
Q

What is the primary classification of lymphomas?

A
  1. Hodgkin
  2. Non-Hodgkin
29
Q

What are 6 B-cell non-Hodgkin lymphomas?

A
  1. Follicular
  2. Diffuse large B-cell lymphoma -aggressive
  3. CLL/SLL
  4. Mantle cell
  5. Burkitt
  6. Lymphoblastic leukemia/lymphoma - B or T cell
30
Q

4 Classic lymphoma histology examples

A
  1. DLBCL
  2. Hodgkin - owl eye = Reed Sternberg
  3. Burkitt - starry sky
  4. Follicular - back to back follicles
30
Q

4 Classic lymphoma histology examples

A
  1. DLBCL
  2. Hodgkin - owl eye = Reed Sternberg
  3. Burkitt - starry sky
  4. Follicular - back to back follicles
31
Q

Tumor staging

A

Stage II - >1 region (noncontiguous)
Stage III - above and below diaphragm
Stage IV - marrow or liver or extranodal

-describe spread of disease
A: absence of B symptoms
B: fever, night sweats, >10% weight loss

32
Q

Leukemia categories

A
33
Q

What defines acute leukemia?

A

> 20% blasts in blood or marrow

34
Q

Dx of AML

A

MPO +
Auer Rods
Adults

35
Q

Signs of Myeloid Neoplasms

A
  1. failed hematopoiesis
  2. cell underproduction
36
Q

Translocations to know

A
  1. t(8;14) most common in Burkitt lymphoma due to overexpression c-myc
  2. t(9;22) CML, misregulation of a tyrosine kinase (Bcr-Abl) - tx = Imatinib
  3. t(14;18) - follicular lymphoma - overexpression Bcl-2 (anti-apoptotic)
  4. t(15;17) - acute promyleocytic leukemia - fusion of retinoic acid receptor (RAR alpha) to PML (a tumor suppressor), TX = tretinoin, ATRA, all-trans retinoic acid)
37
Q

Key differences b/w pediatric and adult tumors

A
38
Q

What is a decrease in CD16 indicate?

A

Left shift - immature cells have decreased Fc receptors, like CD16

39
Q

What is CD34 a marker for?

A

Expressed by HSCs

40
Q

What cell type might be in excess w/ Hodgkin’s Lymphoma?

A

Eosinophils, which is increased by Il-5 production

41
Q

When is basophilia seen?

A

CML

42
Q

What does TdT+ tell us?

A

There are lymphoblasts in the blood which is characteristic of B-ALL (most common) and T-ALL
-is a DNA polymerase, so present in nucleus
-absent in myeloid blasts and mature lymphocytes

43
Q

What does MPO+ indicate in blood?

A

=myeloperoxidase
-indicative of AML
-in structure called Auer rods

44
Q

ALL

A

TdT+
-most common in kids
-assoc w/ Down Syndrome after 5 yo
-can be B-ALL (more common, express CD10, CD19, CD20) or T-ALL (don’t need to know)

45
Q

B-ALL markers and genetic abnormalities

A

TdT+
CD10, CD19, CD20
t(12;21) - more common, good px, in 25% B-ALL
t(9;22)- more common in adults, poor px (BCL-ABL = Philadelphia chromosome)

46
Q

T-ALL markers and genetics

A

TdT+
CD2-CD8 (blasts do not express CD10)
-presents at Thymic mass (mediastinal)
-presents in Teenager
-call it acute lymphoblastic lymphoma b/c it’s a mass

47
Q

APL

A

=acute promyelocytic leukemia, a type of AML
t(15;17) -> PML/RARalpha (retinoic acid) receptor is disrupted -> promyelocytes accumulate, which have Auer rods and are a risk for DIC
-give ATRA to cause blasts to mature

48
Q

What is ATRA?

A

=all trans retinoic acid (derivative of vitamin A) cause blasts to mature and alleviate leukemic burden in pt

49
Q

Acute monocytic leukemia

A

=proliferation of monoblasts that infiltrate the gums and lack MPO

50
Q

Acute megakaryoblastic leukemia

A

=proliferation of megakaryolbasts - lack MPO
-assoc w/ Down syndrome (before age 5) (compare w/ associated after age 5 w/ ALL)

51
Q

What are characteristics of myelodysplastic syndromes?

A

Cytopenias w/ hypercellular bone marrow
-abnorm maturation w/ increased blasts (<20%)
-most pts die from infection or bleeding
-may progress to acute leukemia (>20% blasts)

52
Q

Chronic leukemia characteristics

A

=neoplastic proliferation of mature circulating lymphocytes
-high WBC
-older adults, asymptomatic
-smudge cells
-immunoglobulinemia

53
Q

CLL

A

=neoplastic proliferation of naive B-cells
-cells co-express CD5 and CD20
-increased lymphocytes and smudge cells (splattered-looking cell)
-> generalized lymphadenopathy which is called small lymphocytic lymphoma

54
Q

3 Complications of CLL

A
  1. hypogammaglobulinemia (low Ig) -> primary cause of death is infection
  2. autoimmune hemolytic anemia
  3. transformation to diffuse large B-cell lymphoma
55
Q

Hairy Cell Leukemia

A

=neoplastic proliferation of mature B cells
-characterized by hairy cytoplasmic processes
-cells are TRAP+ (enzyme)
-splenomegaly (red pulp)
-absent lymphoadenopathy
dry tap w/ BM aspiration

*TRAP: TRAP+, trapped in red pulp, trapped in BM, b/c they’re trapped, cannot go to expected place, a lymphnode

56
Q

TX for Hairy Cell Leukemia

A

Drug = 2-CDA
-adenosine deaminase inhibitor
adenosine accumulates to toxic levels in neoplastic B cells, killing them

57
Q

ATLL

A

=Adult T-cell leukemia
=neoplastic proliferation of mature DC4+ T-cells
-assoc w/ HTLV-1 (human T cell leukemia virus 1) - more often seen in Japan and Caribbean

58
Q

ATLL Clinical Features

A
  1. Rash - T-cells like to go to skin, can differentiate from multple myeloma
  2. Generalized LAD (lymphadenopathy) w/ HSM (hepatosplenomegaly)
  3. lytic bone lesions w/ hypercalcemia (seen in multiple myeloma too)
59
Q

What is Sezary syndrome?

A

Seen when mycosis fungoides (neoplastic prolif of mature CD4+ T cells) spreads from skin to blood
-> characteristic lymphocytes w/ cerebriform nuclei (Sezary cells)

MCC (44 decks)

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