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Heme > Markers > Flashcards

Markers Flashcards

1
Q

Name the normal B cell immunophenotype.

A

CD45, CD79a, CD20, IgG kappa or IgG lambda

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2
Q

Name the normal T cell immunophenotype.

A

CD45, TCR (CD3), CD7, CD4 or CD8

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3
Q

What does a “foward scatter” tell you about cells?

A

cell size

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4
Q

What does a “side scatter” tell you about cells?

A

internal granules or segmented nuclei

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5
Q

What is CD34?

A

hematopoietic stem cell marker (blasts)

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6
Q

What is CD33?

A

granulocyte marker

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7
Q

What are the 3 major categories of hematological malignancies?

A

Acute leukemias
Myeloproliferative diseases
Myelodysplasias

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8
Q

What cells are rapidly proliferating in acute leukemias?

A

blasts

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9
Q

What cells are rapidly proliferating in myeloproliferative diseases?

A

a similar clone proliferates and differentiates leading to an increase in some type of peripheral blood cell

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10
Q

What cells are rapidly proliferating in myelodysplasias?

A

a similar clone proliferates and differentiates yielding abnormal blood cell production (low count of some blood cells and abnormal appearance of those cell types)

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11
Q

What cells are just CD34+?

A

blasts

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12
Q

What cells are CD34+, CD33+?

A

myeloid blasts

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13
Q

What cells are Tdt+, CD10+?

A

lymphoid blasts

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14
Q

What cells are CD19+, CD20+?

A

mature lymphocytes/lymphoma

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15
Q

What are the 3 clonal proliferations which we can call acute leukemias even if blast cell count is under 20%?

A

t(8,21) RUNX1-RUNX1T1
inv(16) CBFB-MYH11
t(15,17) PML-RARA

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16
Q

What mutation is found in acute promyelocytic leukemia?

A

t(15;17) PML-RARA

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17
Q

What morphology is unique to APL?

A

big blasts, cleaved “bat wing” nuclei, many cytoplasmic granules, and Auer rods

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18
Q

What is the immunophenotype of APL?

A

Weak/absent CD34, HLA-DR, CD13+, CD33+

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19
Q

AML with t(8,21) leads to what affect?

A

dominant negative repression of myeloid maturation

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20
Q

What morphology is unique to AML with t(8,21)?

A

some maturation of myelocytes, occasional Auer Rods

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21
Q

What is the immunophenotype of AML with t(8,21)?

A

CD34+, HLA-DR+, CD13+, weak CD33

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22
Q

How does AML with inv(16) or t(16,16) relate to AML with t(8,21)?

A

both involve “core binding factor”

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23
Q

What morphology is unique to AML with inv(16)?

A

mixed granulocyte-monocyte features and increased eosinophils in the blood

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24
Q

What is the immunophenotype of AML with inv(16)?

A

CD34+, CD117+ (blasts), CD13+, CD33+ (granulocytes), CD14+, CD11b+ (monocytes)

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25
Q

What is the immunophenotype of AML with normal cytogenetics and complex phenotype?

A

CD34+, CD117+ (blasts), CD 33+ (typically)

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26
Q

t(9;22) is associated with what? Goor or bad prognosis?

A

BCR-ABL1 in ALL (bad prognosis)

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27
Q

What is the immunophenotpe of ALL with t(9;22)?

A

CD10+, CD19+, TdT+

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28
Q

t(v;11q23) is associated with what? Good or bad prognosis?

A

MLL rearranged in ALL (bad prognosis)

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29
Q

What is the immunophenotype of ALL with t(v;11q23)?

A

CD10-, CD19+, TdT+

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30
Q

t(12;21) is associated with what? Good or bad prognosis?

A

TEL-AML1 (ETV6-RUNX1) in ALL (good prognosis)

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31
Q

What is the immunophenotype of ALL with T(12;21)?

A

TdT+, CD34+, CD10+, CD20-

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32
Q

Does hyperdiploid in ALL a good or bad prognosis?

A

good prognosis

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33
Q

T-ALL is associated with what?

A

translocation of an oncogene to one of the 3 T-cell receptor promoters

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34
Q

What is the immunophenotype of T-ALL?

A

TdT+, CD3+, CD5+ (and perhaps myeloid or B-cell antigens as well)

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35
Q

What is the morphology of mastocytosis?

A

aggregates of bland looking cells, round or spindle shaped, sometimes with eosinophilia

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36
Q

What is the immunophenotype of mastocytosis?

A

Tryptase, CD117 (C-KIT), CD25

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37
Q

What is the immunophenotype of B cell precursors in the bone marrow (early)?

A

TdT+, CD10+

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38
Q

What is the immunophenotype of B cell precursors in the bone marrow (late)?

A

TdT+, CD10+, CD19+, CD20+

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39
Q

What is the immunophenotype of Peripheral (mature) or Memory B cells: ?

A

CD19+, CD20+

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40
Q

What is the immunophenotype of B cells in germinal centers?

A

CD10+ (some), CD19+, CD20+

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41
Q

What is the immunophenotype of plasma cells?

A

CD20-, CD38+, CD138+

42
Q

Name the 3 Ig promoters and what chromosomes they are located on.

A 
IgH (14q32)
Ig lambda (22q11)
Ig kappa (2p12)
43
Q

What types of cells are proliferated in CLL?

A

CLL is thought to be derived from the most mature forms of B-cells, or inactive “memory B-cells”.

44
Q

Anatomic distribution of the malignant cells in CLL?

A

Peripheral blood> Lymph nodes (marginal zone) > Bone Marrow

45
Q

The appearance of >30% of what cells in a blood smear of CLL is a good prognosis?

A

smudge cells

46
Q

What peripheral blood smear finding is a bad sign in CLL?

A

Increasing fraction of prolymphocytes

47
Q

What are the two findings in a lymph node of someone with CLL?

A

1) “Pseudofollicular” (collections of slightly larger cells which are undergoing DNA synthesis and mitosis)
2) Effacement of normal architecture

48
Q

What is the normal immunophenotype of someone with CLL?

A

Light Chain Restricted (κ or λ), CD5+, CD23+, CD20 weak

49
Q

What immunophenotype markers are associated with a bad prognosis in CLL?

A

ZAP70+ and CD38+ (markers of somatic hypermutation)

50
Q

What are the most common genetic abnormalities seen in CLL? Which one is good, which one is bad?

A

80% show abnormalities by FISH

1) del13q14.3 (if this only, good prognosis)
2) trisomy 12
3) del11q22-23
4) del17p13 (p53 region is BAD prognosis))

51
Q

What are the 2 major differences between CLL and MCL?

A

1) NO proliferation centers in they lymph nodes of MCL patients
2) MCL patients are CD23-, CD20 STRONG

52
Q

What genetic abnormality is found in MCL?

A

t(11;14)(q13;q32) is a fusion protein of IgH and the oncogene Cyclin D1

53
Q

What is the consequence of the t(11;14)(q13;q32) in MCL?

A

This leads to overexpression of cyclin D1 which pushes the cell through the cell cycle (G1 phase to S-phase).

54
Q

Anatomic distribution of the malignant cells in MCL?

A

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

55
Q

What lymph node findings are unique in MCL?

A

o Usually homogeneous effacement
o “Starry sky”
o Ki-67 immunostain shows increased mitotic rate

56
Q

What is the immunophenotype of MCL?

A

Light Chain Restricted (κ or λ), CD5+, CD23-, CD20 STRONG

57
Q

What are the two forms of plasma cell neoplasm he discussed?

A

1) Monoclonal gammopathy of uncertain significance (MGUS)

2) Multiple myeloma (mean survival 3-4 years)

58
Q

Severe form of plasma cell neoplasms present with what?

A

multiple lytic bone lesions (plasma cell myeloma); pain, fractures, renal failure

59
Q

Anatomic distribution of the malignant cells in plasma cell neoplasms?

A

Bone marrow»peripheral blood

60
Q

What would you expect to see in a peripheral smear of someone with MGUS (mild plasma cell neoplasm)?

A

Rouleaux (little stacks of RBCs)

61
Q

What is the immunophenotype of plasma cell neoplasm?

A

Light Chain Restricted (κ or λ), CD38+++, CD138+++, CD19-, CD20-

62
Q

What are the 2 major genetic findings in plasma cell neoplasms?

A

1) Translocation of IgH to various oncogenes (FISH) in about 2/3 of cases.
2) Trisomies of ODD NUMBERED chromosomes

63
Q

List the 5 genetic abnormalities in plasma cell neoplasms that are poor prognostic markers.

A 
Serum beta 2 microglobulin
t(4;14)   FGFR3   
t(14;16)  C-MAF
t(14;20)  MAFB
del 17p (p53 region)
64
Q

Grade in follicular lymphoma (FL) is dependent on finding what cells in bone marrow?

A

More centroblasts (larger, less-dark ones), higher grade!

65
Q

Anatomic distribution of the malignant cells in FL?

A

Lymph nodes > Bone marrow (40-70%) > Peripheral blood (maybe)

66
Q

What does a lymph node look like in someone with FL?

A 
Lymph node (enlarged with many follicles)
o	No polarity (large-to-small cells)
o	No tingible body macrophages
o	Fewer mitotic figures than normal
o	Germinal center stains for Bcl-2
67
Q

What is the immunophenotype of someone with FL?

A

CD19+, CD20+. CD10+ (60%). BCL-2+ (90%). BCL-6+ (85%)

68
Q

What is the major genetic finding in FL?

A

t(14;18)(q32;q21)+ in >85% (BCL2 gene)

69
Q

What is the consequence of t(14;18)(q32;q21)+ in FL?

A

Failure of germinal center B-cells to apoptose (because they overexpress an anti-apoptotic protein, Bcl-2) because centroblasts translocate Bcl-2 to an IgH promoter.

70
Q

40% of patients with Diffuse large B-cell lymphoma present with what?

A

Extranodal disease (GI tract, bone marrow, other)

71
Q

What is the immunophenotype of Diffuse large B-cell lymphoma?

A

CD19+, CD20+. CD10+ (30-60%)

72
Q

What are some genetic findings in Diffuse large B-cell lymphoma?

A
  • t(v, 3q27)(v, BCL-6) in ~30%;
  • t(14;18) in 20-30%
  • Multiple other translocations/deletions can occur
73
Q

Describe the typical patient presenting with Hodgkin lymphoma.

A
  • Males, age 30-50
  • Localized or diffuse adenopathy
  • Often involvement of cervical, mediastinal, or abdominal lymph nodes, and/or spleen
74
Q

What are the two subtypes of Hodgkin lymphoma (and what are their prognoses)?

A

Classical Hodgkin Lymphoma (curable with chemo/RT; 97% 10 yr survival rate)
Nodular Lymphocyte Predominant Hodgkin Lymphoma (80% 10 yr survival, 3-5% progress to diffuse large cell B-cell lymphoma)

75
Q

What cells are specific to Hodgkin lymphoma?

A

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

76
Q

An HL lymph node that is criss-crossed by fibrous bands is in what morphological subtype?

A

Nodular sclerosis pattern

77
Q

An HL lymph node that is NOT criss-crossed by fibrous bands is in what morphological subtype?

A

Mixed cellularity pattern

78
Q

An HL lymph node thats background is mostly lymphocytes is in what morphological subtype?

A

Lymphocyte rich pattern

79
Q

An HL lymph node thats background is mostly R/S cells is in what morphological subtype?

A

Lymphocyte depleted pattern

80
Q

A lymph node in NLPHD has what findings?

A
  • Nodular appearance
  • Mummified R/S cells (smaller, less prominent nucleoli)
  • “Popcorn-looking” L&H cells
81
Q

What is the immunophenotype of classical HL?

A

R/S cells are too fragile for Flow cytometry

CD30+ (>90%), CD15+ (>80%), Pax5+ (>90%) (B-cell transcription factor), CD20-/wk

82
Q

What is the immunophenotype of NLPHD?

A

CD30- (>80%), CD15- (>100%), Pax5+ (>95%), CD20+ (>95%), T cells surround R/S cells

83
Q

Why does HL occur?

A

Reed Sternberg cells are B-cells which went into germinal centers expecting to die, but instead acquired both anti-apoptotic mutations or anti-apoptotic genes from EBV (NFkB expression) and disguised themselves by disabling IgH expression.

84
Q

How does the molecular pathology behind HL and NLPHD differ?

A

Generally the same as classical, but NO disabling of IgH expression

85
Q

What is the immunophenotypic findings of NK cells?

A

CD3+ cytoplasmic

86
Q

What is the immunophenotypic findings of gamma-delta T-cells?

A

CD3+ surface

87
Q

What is the immunophenotype of developing T cells?

A

TdT+ and CD7+

88
Q

What is the immunophenotype of mature T cells?

A

CD3+ (surface), CD7+ and CD4+ or CD8+

89
Q

True or false: almost every T cell lymphoma looks the same.

A

FALSE: T cell lymphomas do not have any apparent trends (presentation is highly variable!!!)

90
Q

What is the one test you can perform if you expect a T cell lymphoma?

A

Clonality can be demonstrated by PCR-based studies of the TCR gene.

91
Q

What T cell malignancy is immunophenotypically like a helper T cell and has many skin lesions?

A

Mycosis fungoides

92
Q

If Mycosis fungoides begins to involve the blood, what do you call it?

A

Sezary syndrome

93
Q

What are the major presenting features of Angioimmunoblastic T-Cell Lymphoma?

A
  • Rapidly progressive critical illness
  • Diffuse lymphadenopathy
  • Hepatosplenomegaly
  • Skin rash
  • Cold autoimmune hemolytic anemia
  • Evidence of immunocompromise
94
Q

Angioimmunoblastic T-Cell Lymphoma is a proliferation of what type of cells? (immunophenotype)

A

T-follicular helper cells (CD3+, CD4+, CD10+)

95
Q

What two strange immunophenotypic findings are characteristic of Angioimmunoblastic T-Cell Lymphoma and must be stained for?

A

1) they express a marker usually seen on germinal center B-cells, CD10+.
2) Residual follicular dendritic cell (FDC) frameworks still present and stain for CD21+

96
Q

What drugs should NOT be used to treat Angioimmunoblastic T-Cell Lymphoma?

A

Standard chemotherapeutic agents have a high morbidity rate in AILT because their immunocompromising side effects are compounded by the immunocompromise inherent to the disease itself.

97
Q

What is the typical clinical presentation of someone with Peripheral T Cell Lymphoma NOS?

A
  • Diffuse lymphadenopathy
  • B symptoms (fever, night sweats, weight loss)
  • Paraneoplastic features
  • Eosinophilia
  • Pruritis
  • Hemolytic anemia
98
Q

True or false: Peripheral T Cell Lymphoma NOS is an aggressive disease.

A

true! 5 year survival only 20-30%

99
Q

What are the expected lymph node features in Peripheral T Cell Lymphoma NOS?

A
  • Expanded Paracortex

* Normal architecture is effaced

100
Q

Describe the immunophenotype of Peripheral T Cell Lymphoma NOS. (even variants and unexpected findings)

A

What’s expected: CD3, 5, 7, and 4 or 8
What’s usually seen: Loss of one or more of the above
Variants: “Double positives” (CD4+, CD8+)
Unexpected markers: CD20 (B-cell), CD56 (macrophage/monocyte), CD30 (R/S cell)

Heme (10 decks)

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