Hematologic Malignancies 3 Flashcards

(58 cards)

1
Q

Immunophenotype of B-cell precursors in bone marrow

A
  1. TdT+
  2. CD10+
  3. CD19+
  4. CD20+
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2
Q

Immunophenotype of naive B-cells in lymphoid tissues

A
  1. TdT+
  2. CD10+ (in germinal center)
  3. CD19+
  4. CD20+
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3
Q

Immunophenotype of B-cells in follicular area (centroblasts and centrocytes)

A
  1. CD19+
  2. CD20+
  3. CD10+
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4
Q

Immunophenotype of plasma cell

A
  1. CD38+
  2. CD138+
  3. CD20-
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5
Q

Location of Ig promoters

A

IgH (14q32)

Ig lambda (22q11)

Ig kappa (2p12)

–> oncogene translocation to these areas causes overexpression of oncogene product

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6
Q

Cells thought to cause CLL

A

Memory B-cells found in the marginal zone of the lymph node

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7
Q

Clinical presentation of chronic lymphocytic leukemia/lymphoma

A

Lymphocytosis in older males –> high familial incidence

Unexplained recurring infection due to hypogammaglobulinemia (infection is the most common cause of death)

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8
Q

Sites involved in CLL/SLL

A

Peripheral blood > bone marrow, lymph nodes

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9
Q

Peripheral blood morphology of CLL/SLL

A

Small lymphocytes, little cytoplasm and mature dense chromatin –> monomorphic appearance

Smudge cells

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10
Q

Lymph node morphology of CLL/SLL

A

“Pseudofollicular” –> collections of slightly larger cells undergoing DNA synthesis

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11
Q

Cytogenetics of CLL/SLL

A

Ranked by prognosis

del13 > trisomy 12 > del11, del17 (p53 region… bad)

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12
Q

Immunophenotype of CLL/SLL

A
  1. ZAP-70 and CD38 expression (bad) –> Markers of somatic hypermutation status
  2. Light chain restriced (kappa or lambda)
  3. CD20 weak
  4. CD5+
  5. CD23+
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13
Q

Clinical presentation of mantle cell lymphoma

A

Lymphadenopathy and/or lymphocytosis in older males –> can look clinically like CLL

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14
Q

Sites involved in mantle cell lymphoma

A

Lymph nodes > bone marrow, spleen, peripheral blood, GI tract

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15
Q

Morphology of mantle cell lymphoma

A

Peripheral blood smear: small lymphocytes, little cytoplasm; “smudge” cells

Lymph node: Usually homogenous effacement, ‘starry sky’

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16
Q

Immunophenotype of of mantle cell lymphoma

A

Similair to CLL

  1. Light chain restricted (kappa or lambda)
  2. CD5+
  3. CD20 strong
  4. CD23-
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17
Q

Cytogenetics of of mantle cell lymphoma

A

t(11;14)(IgH;CyclinD1) –> overepxression of cyclin D1 pushes the cell through the cell cycle G1 to S

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18
Q

Clincal presentation of Burkitt lymphoma (sporadic)

A

Abdominal/pelvic mass in sporadic form

–> Dysfunctional memory B cells

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19
Q

Sites involved in Burkitt lymphoma (sporadic)

A
  1. Ileo-cecal area
  2. Ovaries
  3. Kidneys
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20
Q

Morphology of Burkitt lymphoma (sporadic)

A

Cytology: Intermediate sized cells with basophilic vacuolated cytoplasm

Tissue: Usually homogenous effacement, high growth rate, ‘starry sky’

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21
Q

Clinical presentation of Burkitt lymphoma (endemic)

A

Jaw/facial bone mass in child (age 4-7) in a p. falciparum malaria-endemic area (Ghana, Papua New Guinea)

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22
Q

Cytogenetics of Burkitt lymphoma

A

Translocation of an oncogene (MYC on 8q24) to an Ig promoter

t8;14

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23
Q

Morphology of Burkitt lymphoma (endemic)

A

Same as sporadic

Cytology: Intermediate sized cells with basophilic vacuolated cytoplasm

Tissue: Usually homogenous effacement, high growth rate, ‘starry sky’

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24
Q

Immunophenotype of Burkitt lymphoma (sporadic or endemic)

A

Typical B-cell markers (CD10, CD19, CD20)

CD22+, CD79+, Ki-67+

25
Clinical presentation of plasma cell neosplams
Older individuals Mild forms: Asymptomatic lab findings (monoclonal gammopathy of uncertain significance; MGUS) Severe forms: multiple lytic bone lesions, pain, fractures, renal failure
26
Sites involved in plasma cell neoplasms
Bone marrow \>\> peripheral blood
27
Morphology of peripheral smear in plasma cell neoplasms
Rouleaux red cells
28
Lab findings in plasma cell neoplasms
Mild forms: increased total protein, Rouleaux noted on peripheral smear M spike of monoclonal Ig will be seen on electrophoresis
29
Morphology of bone marrow in plasma cell neoplasms
Plasma cells show a lot of cytoplasm and nucleus off center
30
Immunophenotype of plasma cell neoplasms
1. CD38+++ 2. CD138+++ 3. CD19- 4. CD20- 5. Light chain restricted
31
Cytogenetics of plasma cell neoplasms
Translocation of IgH to various oncogenes (FISH) in about 2/3 of cases Trisomies (hyperploidy) of ODD NUMBERED CHROMOSOMES are common
32
Clinical course of plasma cell neoplasms
MGUS: 1%/yr progress to MM Multiple myeloma: median survival 3-4 years
33
Key negative clinical predictors of plasma cell neoplasms
1. Serum beta 2 microglobulin 2. t(4;14) FGFR3 3. t(14;16) C-MAF 4. t(14;20)MAFB 5. del 17p (p53 region)
34
Histological appearance of lymph nodes in follicular lymphoma
No polarity (large to small cells) No tingible body macrophages Fewer mitotic figures than normal Bcl-2 immunostains can be used to distinguish follicular lymphoma from reactive follicular hyperplasia
35
Clinical presentaion of follicular lymphoma
Lymphadenopathy in older individuals Can be otherwise asymptomatic Bone marrow involved in 40-70% of cases Can involve peripheral blood
36
Immunophenotype of follicular lymphoma
1. CD19+ 2. CD20+ 3. CD10+ (60%) 4. Bcl-2+ (90%) 5. Bcl-6+ (85%)
37
Cytogenetics of follicular lymphoma
t(14;18)+ in \>85% Centroblast translocates Bcl2 to IgH prmoter region --\> enlarged lymph node with many follicles
38
Grading of follicular lymphoma
Graded by presence of large cells (centroblasts) Grade 1 --\> mostly centrocytes Grade 2 --\> mix of centrocytes and centroblasts Grade 3 --\> mostly centroblasts
39
Clinical presentation of diffuse large B-cell lymphoma
Old people with rapid growing adenopathy
40
Immunophenotype of diffuse large B-cell lymphoma
1. CD19+ 2. CD20+ 3. CD10+ (30-60%)
41
Cytogenetics of diffuse large B-cell lymphoma
1. t(v;3)(v;Bcl-6) in ~30% 2. t(14;18) in 20-30%
42
Clincal presentation of Hodgkin lymphoma
1. Males age 30-50 2. Localized or diffuse adenopathy 3. Often with involvement of cervical, mediastinal, or abdominal lymph nodes, and/or spleen
43
Morphology of Hodgkin lymphoma
Reed/Sternberg cells --\> Large lymphoid cells with mono- or bi-nucleate appearance, huge eosinophilic nucleoli; overall horeshoe shape is likely Diverse background cells
44
Morphology of Hodgkin lymphoma
1. Normal lymph node architecture 2. Can be criss-crossed by fibrous bands 3. Can show background that's mostly lymphocytes 4. Can show large number of Reed/Sternberg cells
45
Immunophenotype of **classical** Hodgkin lyphoma
Reed/Sternberg cells don't express surface Ig 1. CD30+ 2. CD15+ 3. Pax5+ 4. CD20-
46
Morphology of nodular lymphocyte predominant Hodgkin lymphoma
Nodular with mostly lymphocytes in the background "popcorn" cells (lympho-histiocytes) in place of Reed/Sternberg cells
47
Immunophenotype of nodular lymphocyte predominant Hodgkin lymphoma
Lympho-histiiocyte cells 1. CD30- (80%) 2. CD15- (100%) 3. Pax5+ (\>95%) 4. CD20+ (\>95%) 5. T-cells surround the R/S cells
48
Indicated site of ALK+ anaplastic large cell lymphoma
Lymph nodes primarily Bone marrow, bone cortex, liver, soft tissues also
49
Indicated site of angio-immunoblastic T-cell lymphoma
Lymph nodes
50
Indicated site of mycosis fungoides
Primarily skin T-cells express CD4 and CD3, but not the other T-cell markers, so diagnosis can be made on lacking T-cell markers
51
Indicated site of sezary syndrome
Subset of mycosis fungiodes --\> signifcant peripheral blood involvment Peripheral blood and skin are primary sites
52
Clincal presentation of mycosis fungoides
Patchy, flat red skin lesions that progress to thick, psoriasis-like or ulcerated lesions Usually in elderly patients
53
Morphology of mycosis fungiodes
Cytology: normal size lymphocytes with indented nuclei Tissue: Bland looking lymphocytes that invade the epidermis Bloodstream: bland lymphocytes with "cerebriform" nuclei
54
Immunophenotype and cytogenetics of mycosis fungoides
CD3, CD4, CD5 + Clonal re-arrangement of T-cell receptor gene
55
Clinical presentation of peripheral T-cell lymphoma NOS
1. Diffuse lymphadenopathy 2. B symptoms (fever, night sweats, weight loss) 3. Paraneoplastic features (eosinophilia, pruritis, hemolytic anemia,
56
Lymph node Morphology of peripheral T-cell lymphoma NOS
Expanded paracortex Effacement of normal architecture
57
Immunophenotype of peripheral T-cell lymphoma NOS
Usually contain CD3, CD5, CD7, CD4, and CD8 In malignancy one or more of these is absent Can also show CD20 (B-cell marker), CD56 (macrophage/monocyte marker), CD30 (R/S cell marker)
58
1. Genetics of peripheral T-cell lymphoma NOS
Complex karyotype Multiple chromosomal gains and losses Multiple chromosomal deletions