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HEME > Hematologic Malignancies 3 > Flashcards

Hematologic Malignancies 3 Flashcards

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
Q

Clinical presentation of plasma cell neosplams

A

Older individuals

Mild forms: Asymptomatic lab findings (monoclonal gammopathy of uncertain significance; MGUS)

Severe forms: multiple lytic bone lesions, pain, fractures, renal failure

26
Q

Sites involved in plasma cell neoplasms

A

Bone marrow >> peripheral blood

27
Q

Morphology of peripheral smear in plasma cell neoplasms

A

Rouleaux red cells

28
Q

Lab findings in plasma cell neoplasms

A

Mild forms: increased total protein, Rouleaux noted on peripheral smear

M spike of monoclonal Ig will be seen on electrophoresis

29
Q

Morphology of bone marrow in plasma cell neoplasms

A

Plasma cells show a lot of cytoplasm and nucleus off center

30
Q

Immunophenotype of plasma cell neoplasms

A
  1. CD38+++
  2. CD138+++
  3. CD19-
  4. CD20-
  5. Light chain restricted
31
Q

Cytogenetics of plasma cell neoplasms

A

Translocation of IgH to various oncogenes (FISH) in about 2/3 of cases

Trisomies (hyperploidy) of ODD NUMBERED CHROMOSOMES are common

32
Q

Clinical course of plasma cell neoplasms

A

MGUS: 1%/yr progress to MM

Multiple myeloma: median survival 3-4 years

33
Q

Key negative clinical predictors of plasma cell neoplasms

A
  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
Q

Histological appearance of lymph nodes in follicular lymphoma

A

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
Q

Clinical presentaion of follicular lymphoma

A

Lymphadenopathy in older individuals

Can be otherwise asymptomatic

Bone marrow involved in 40-70% of cases

Can involve peripheral blood

36
Q

Immunophenotype of follicular lymphoma

A
  1. CD19+
  2. CD20+
  3. CD10+ (60%)
  4. Bcl-2+ (90%)
  5. Bcl-6+ (85%)
37
Q

Cytogenetics of follicular lymphoma

A

t(14;18)+ in >85%

Centroblast translocates Bcl2 to IgH prmoter region –> enlarged lymph node with many follicles

38
Q

Grading of follicular lymphoma

A

Graded by presence of large cells (centroblasts)

Grade 1 –> mostly centrocytes

Grade 2 –> mix of centrocytes and centroblasts

Grade 3 –> mostly centroblasts

39
Q

Clinical presentation of diffuse large B-cell lymphoma

A

Old people with rapid growing adenopathy

40
Q

Immunophenotype of diffuse large B-cell lymphoma

A
  1. CD19+
  2. CD20+
  3. CD10+ (30-60%)
41
Q

Cytogenetics of diffuse large B-cell lymphoma

A
  1. t(v;3)(v;Bcl-6) in ~30%
  2. t(14;18) in 20-30%
42
Q

Clincal presentation of Hodgkin lymphoma

A
  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
Q

Morphology of Hodgkin lymphoma

A

Reed/Sternberg cells –> Large lymphoid cells with mono- or bi-nucleate appearance, huge eosinophilic nucleoli; overall horeshoe shape is likely

Diverse background cells

44
Q

Morphology of Hodgkin lymphoma

A
  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
Q

Immunophenotype of classical Hodgkin lyphoma

A

Reed/Sternberg cells don’t express surface Ig

  1. CD30+
  2. CD15+
  3. Pax5+
  4. CD20-
46
Q

Morphology of nodular lymphocyte predominant Hodgkin lymphoma

A

Nodular with mostly lymphocytes in the background

“popcorn” cells (lympho-histiocytes) in place of Reed/Sternberg cells

47
Q

Immunophenotype of nodular lymphocyte predominant Hodgkin lymphoma

A

Lympho-histiiocyte cells

  1. CD30- (80%)
  2. CD15- (100%)
  3. Pax5+ (>95%)
  4. CD20+ (>95%)
  5. T-cells surround the R/S cells
48
Q

Indicated site of ALK+ anaplastic large cell lymphoma

A

Lymph nodes primarily

Bone marrow, bone cortex, liver, soft tissues also

49
Q

Indicated site of angio-immunoblastic T-cell lymphoma

A

Lymph nodes

50
Q

Indicated site of mycosis fungoides

A

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
Q

Indicated site of sezary syndrome

A

Subset of mycosis fungiodes –> signifcant peripheral blood involvment

Peripheral blood and skin are primary sites

52
Q

Clincal presentation of mycosis fungoides

A

Patchy, flat red skin lesions that progress to thick, psoriasis-like or ulcerated lesions

Usually in elderly patients

53
Q

Morphology of mycosis fungiodes

A

Cytology: normal size lymphocytes with indented nuclei

Tissue: Bland looking lymphocytes that invade the epidermis

Bloodstream: bland lymphocytes with “cerebriform” nuclei

54
Q

Immunophenotype and cytogenetics of mycosis fungoides

A

CD3, CD4, CD5 +

Clonal re-arrangement of T-cell receptor gene

55
Q

Clinical presentation of peripheral T-cell lymphoma NOS

A
  1. Diffuse lymphadenopathy
  2. B symptoms (fever, night sweats, weight loss)
  3. Paraneoplastic features (eosinophilia, pruritis, hemolytic anemia,
56
Q

Lymph node Morphology of peripheral T-cell lymphoma NOS

A

Expanded paracortex

Effacement of normal architecture

57
Q

Immunophenotype of peripheral T-cell lymphoma NOS

A

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
Q
  1. Genetics of peripheral T-cell lymphoma NOS
A

Complex karyotype

Multiple chromosomal gains and losses

Multiple chromosomal deletions

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