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Boards.CP.Heme > T Cell Neoplasms > Flashcards

T Cell Neoplasms Flashcards

1
Q

Angioimmunoblastic T cell lymphoma (AITCL):

  • Caused by
  • Population
A

EBV associated

-Older adults

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2
Q

Enteropathy associated T cell lymphoma is strongly associated with what ancestral groups?

A

Welsh and Irish

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3
Q

Hepatosplenic T cell lymphoma (HSTCL):

-Which type is more common in young males?

A

gamma/delta type

  • alpha/beta type
  • female predominance
  • wider age distribution
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4
Q

Angioimmunoblastic T cell lymphoma (AITCL) - Morphology:

-Cellular milieu (5)

A
  • Aggregates of cells w/ clear cytoplasm
  • Immunoblasts
  • Lymphocytes
  • Plasma cells
  • Eosinophils
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5
Q

T cytotoxic LGL leukemia - Immunophenotype:

-Positive for (7)

A
  • CD2
  • CD3
  • CD8
  • CD16
  • CD57
  • granzyme B
  • granzyme M
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6
Q

Adult T cell leukemia/lymphoma (ATCL) - Clinical presentation. (6)

A
  • Lymphadenopathy
  • Hepatosplenomegaly
  • Skin rash
  • HyperCalcemia
  • Lytic bone lesions
  • Visceral involvement (CNS, lungs, GI tract)
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7
Q

Angioimmunoblastic T cell lymphoma (AITCL) - Morphology:

-Architecture (4)

A
  • Diffuse nodal effacement w/prominent postcapillary venules
  • Deposition of PAS+ extracellular material
  • Mixed lymphoid infiltrate
  • Absence of follicles
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8
Q

T cytotoxic LGL leukemia is usually indolent; however aggressive disease is seen in cases with what?

A

CD56+ w/ blastlike morphology

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9
Q

Angioimmunoblastic T cell lymphoma (AITCL) - Clinical presentation:

  • Onset (gradual vs abrupt)
  • Signs/Symptoms (4)
A 
-Abrupt onset
Signs/Symptoms:
-Constitutional (fever, night sweats, weight loss)
-Lymphadenopathy
-Pruritic skin rash
-Pleural effusion
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10
Q

Enteropathy associated T cell lymphoma (EATCL) - Immunophenotype:

  • Positive for (2)
  • Negative for (2)
  • HLA genotype
A 
Positive:
-CD3
-CD30
Negative
-CD48
-CD

HLADQA10501, DQB1*0201

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11
Q

Cutaneous T cell lymphoma (CTCL) that involves lymph nodes is called what?

A

Mycosis Fungoides (MF)

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12
Q

Anaplastic Large cell lymphoma (ALCL) - Morphology:

  • Architecture
  • Cell size
  • Where do anaplastic cells cluster?
A
  • Diffuse proliferation
  • Large lymps; some of which are anaplastic
  • Anaplastic cells cluster near Blood Vessels
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13
Q

Anaplastic Large cell lymphoma (ALCL) - Prognosis factor:

-Best prognosis

A

Alk expression
-Alk+ has best prognosis

*WHO classification has separate categories for Alk+ and Alk- ALCL

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14
Q

Cutaneous T cell lymphoma (CTCL) is a ___ T cell neoplasms with ________ growth pattern.

A

Cutaneous T cell lymphoma (CTCL) is a CD4+ T cell neoplasms with Epidermotropic growth pattern.

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15
Q

T cytotoxic LGL leukemia - Immunophenotype:

-Negative for (3)

A
  • CD4
  • CD5 (neg/dim)
  • CD7 (neg/dim)
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16
Q

ALK gene location.

A

Anaplastic Lymphoma Kinase (ALK)

-Chr2p23

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17
Q

What lymphoma arises in patients with longstanding celiac sprue?

  • Often preceded by what?
  • Ancestories (2)
A

Enteropathy associated T cell lymphoma (EATCL)

  • Refractory sprue with mucosal ulceration (ulcerative jejunoileitis)
  • Welsh and Irish
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18
Q

NPM gene location.

A

Nucleophosmin (NPM)

-Chr5q35

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19
Q

Peripheral T cell lymphoma (PTCL) - Immunophenotype:

  • CD4/CD8
  • CD25
  • other features
A
  • CD4+; CD8-
  • CD25-
  • loss of one or several pan T cell markers
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20
Q

T/F: Clonal TCR rearrangement is seen in 90% of ALCL cases.

A

True

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21
Q

Angioimmunoblastic T cell lymphoma (AITCL) - Immunophenotype:

  • Positive for (8)
  • Negative for (1+)
A

Positive:

  • CD2
  • CD3
  • CD4
  • CD5
  • CD7
  • CD10
  • bcl-6
  • CXCL-13
22
Q

T cytotoxic LGL leukemia:

  • Presentation (3)
  • Population
  • Autoimmune association
A 
Presentation
-Neutropenia
-Splenomegaly
-Polyclonal hypergammaglobulinemia
Population: Older males (>60 y/o)
-Rheumatoid Arthritis has higher incidence
23
Q

Angioimmunoblastic T cell lymphoma (AITCL) - Hematologic/Immunologic associations (5)

A
  • Coombs+ autoimmune hemolytic anemia
  • Cold agglutinins
  • Anti-smooth muscle Ab
  • Rheumatoid factor
  • Polyclonal hypergammaglobulinemia
24
Q

Adult T cell leukemia/lymphoma (ATCL) - Immunophenotype:

  • Positive for (5)
  • Negative for (2)
A

Positive:

  • CD2
  • CD3
  • CD4
  • CD5
  • CD25

Negative:

  • CD8
  • CD7 (usually)
25
Q

Large Granular Lymphcytic Leukemia (LGL leukemia):

  • Definition
  • Cell types (2)
A 
Unexplained increase in LGLs
->6 months
->2x10^9 LGLs
Cell types:
-T cytotoxic cells or NK cells
26
Q

What population is at higher risk for Anaplastic Large cell lymphoma (ALCL)?

A

Children and Young Adults

*50% of childhood high grade lymphomas

27
Q

Anaplastic Large cell lymphoma (ALCL) - ALK Expression:

  • Correlates with what translocation
  • Where is it expressed?
A

t(2;5)/NPM-ALK translocation

-Alk is expressed in cytoplasm and nucleus

28
Q

NK cell LGL leukemia:

  • EBV (+/-)
  • Cytogenetics
A
  • EBV- (in contrast to aggressive NK cell leukemia (ANKL) which is EBV+)
  • TCR germline
29
Q

What is the cause of Adult T cell leukemia/lymphoma (ATCL)?

-Lifetime risk?

A

HTLV-1

-5% (7% for males; 3% for females)

30
Q

What are the most common T-cell neoplasms, in decreasing order? (4)

A
  • Peripheral T cell lymphoma (PTCL)
  • Angioimmunoblastic T cell lymphoma (AITCL)
  • Anaplastic Large cell lymphoma (ALCL)
  • Adult T cell leukemia/lymphoma (ATCL)
31
Q

Which has a worse prognosis - Alk- ALCL or PTCL?

32
Q

NK cell LGL leukemia - Immunophenotype:

-Positive for (3)

A
  • CD2
  • CD16
  • CD56
33
Q

Angioimmunoblastic T cell lymphoma (AITCL) - Immunophenotype:
-Negative for (1+)

A

Negative:

  • CD8
  • Loss of one or several pan T cell markers (CD2,3,5,7)
34
Q

Anaplastic Large cell lymphoma (ALCL) - Immunophenotype:

-Positive for (7)

A
  • T cell Ags (CD2,3,5,7)
  • CD4
  • CD30 (membranous/Golgi)
  • CD45
  • Clusterin
  • EMA
  • Myeloid Ags (CD13/CD33)
35
Q

What are the pan T cell markers? (4)

A
  • CD2
  • CD3
  • CD5
  • CD7
36
Q

the t(2;5)/NPM-ALK rearrangement is present in what percentage of cases of ALCL?

37
Q

Nasal type NK/T cell lymphomas (NTNKT):

  • growth pattern
  • EBV (+/-)
  • Population
A
  • Angioinvasive growth pattern
  • EBV+
  • Asians, Central/South America
38
Q

T-cell neoplasms:

  • % of lymphoid neoplasm overall
  • Highest incidence population
A

5% of all lymphoid neoplasms

-Asia has highest incidence

39
Q

Anaplastic Large cell lymphoma (ALCL) - Small Cell Variant - Morphology:
-DDX

A

Composed of Large lymphoid cells
-NOT anaplastic

DDX: Peripheral T cell lymphoma (PTCL)

40
Q

T cytotoxic LGL leukemia - Cytogenetics.

A

TCR rearranged

41
Q

Angioimmunoblastic T cell lymphoma (AITCL):

-What does CD21 show?

A

Hyperplastic follicular dendritic cells

-Highlights the residual follicular dendritic cell meshwork

42
Q

NK cell LGL leukemia - Immunophenotype:

-Negative for (2)

A
  • CD3 (surface)

- CD4

43
Q

Adult T cell leukemia/lymphoma (ATCL) - Morphology. (1)

A

Nuclear irregularity

-Cloverleaf or Flowerlike forms

44
Q

NK cell LGL leukemia - Immunophenotype:

-Variable for (3)

A
  • CD7
  • CD8
  • CD57
45
Q

Describe the cells in Cutaneous T cell lymphoma (CTCL).

A

Small to Large lymphoid cells

-CEREBRIFORM Nuclei

46
Q

NK cell LGL leukemia:

-Presentation (5)

A
  • Neutropenia
  • Anemia
  • Fever
  • Jaundice
  • Hepatosplenomegaly
47
Q

What is the immunophenotype for Follicular T Helper cells (TFH)? (3)

A
  • CD10
  • bcl-6
  • CXCL-13
48
Q

Cutaneous T cell lymphoma (CTCL) involving peripheral blood is called what?

A

Sezary Syndrome

49
Q

What age/population is Aggressive NK cell leukemia (ANKL) more common in?
-What is it associated with?

A

Asians (mean 40 y/o)

-EBV+

50
Q

Peripheral T cell lymphoma (PTCL) - Morphology:

  • Architecture
  • Nuclei
  • Cellular milieu
  • What may be prominent?
A
  • Diffuse, polymorphic, small and large lymphoid cells
  • “Cloverleaf” (multilobate nuclei)
  • Eosinophils, Plasma cells, and/or histiocytes
  • Postcapillary venules may be prominent

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