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Hematopathology > T and NK Cell Neoplasms > Flashcards

T and NK Cell Neoplasms Flashcards

1
Q

LyP clinical

A
  • 12% cutaneous lymphomas
  • chronic, recurrent, self-healing skin dz affecting trunk, extremities, butt
  • papular/nodular lesions, centrally necrotic, up to 2cm in diameter
  • appear in clusters, recur in same region of body
  • regress spontaneously within 4-6 weeks, leaving a hyper-/hypo- pigmented scar
  • may get regional LAD
  • 20% of patients the dz is preceded by, associated with, or followed by another lymphoma: MF, C-ALCL, cHL
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2
Q

LyP Morphology

A
  • early lesions show mainly perivascular/superficial dermal atypical lymphoid cells surrounded by variale #inflammatory cells
  • PMNs within blood vessel lumens nearly a constant feature**
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3
Q

T LBL immunophenotype

A 
CD7 most sensitive
Tdt
CD3 may be cytoplasmic only - specific
CD4/CD8 often coexpressed
CD1a, CD2, CD5, CD7, CD10, myeloid antigens variable

Pitfall: CD79a positivity on IPOX

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4
Q

T LBL genetics

A

Rearrangement T cell receptor loci (alpha/delta, beta, gamma), also IgH frequently
Partners:
Transcription factors: HOX11, HOX11L2, MYC, TAL1, RBTN1, RBTN2, LYL1
Tyrosine kinase: LCK
del(9p) (CDKN2A)
Notch1 mutation

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5
Q

T PLL immunophenotype

A 
Immunophenotype
CD2, CD3, CD7 positive
Tdt and CD1a negative
CD4+/CD8-, CD4+/CD8+ or CD4-/CD8+
Immunohistochemistry 
TCL1
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6
Q

T PLL genetics

A 
Genetics 
Clonal TCR rearrangement
Inv14 or t(14;14) 
TCR with TCL1a, TCL1b
others
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7
Q

T-LGL immunophenotype

A

Immunophenotype
CD3, TCR-a/b, CD7, CD8 positive
CD4-
CD11b, CD57, CD56 variable

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8
Q

T-LGL genetics

A

Genetics
Clonal TCR rearrangement
STAT3 mutations in 40% of cases (NEJM 366:1905, 2012)

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9
Q

CLPD-NK

A

Morphology
Similar to T-LGL Immunophenotype
CD2, CD3 (cytoplasmic), CD7, CD16, CD56, CD57
Surface CD3-negative

Genetics
Germline TCR

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10
Q

Aggressive NK Cell Leukemia

A 
Morphology 
Variable, ranging from LGL-like to blastic large cells
Immunophenotype 
CD2, CD3 (cytoplasmic), CD16, CD56
Surface CD3 negative
EBV+

Genetics
Germline TCR

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11
Q

EBV+ LPD of Childhood

A

Systemic EBV+ T cell LPD of childhood:

  • Chronic active EBV infection
  • Prominent hemophagocytosis
  • EBV+

Hydroa vacciniforme-like lymphoma:
-Cutaneous manifestation of T cell, rarely NK cell lymphoma associated with EBV infection

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12
Q

ATLL Clinical

A
  • Endemic in Japan, Caribbean basin, Central Africa
  • Caused by human retrovirus, human T-cell leukemia virus type 1 (HTLV-1) – serology testing
  • Clinical variants: Acute Lymphomatous, Chronic Smoldering, Hodgkin-like
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13
Q

ATLL Morphology and Genetics

A

Morphology
Very pleomorphic ranging from small cells with irregular nucleoli to large anaplastic cells
Peripheral blood with “flower cells”

Genetics
Clonal TCR rearrangement
Clonal integration HTLV-1

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14
Q

ATLL Immunophenotype

A 
Immunophenotype
CD2, CD3, CD5, CD25 +
CD7-
CD4+, CD8-  (most cases)
FOXP3+ (Treg)
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15
Q

Extranodal NK/T Cell Lymphoma, Nasal Type- Morphology & Genetics

A

Morphology
Diffusely infiltrating cells ranging in size from small to large in size.
Angiocentric and angioinvasive pattern may be present.
Necrosis is often present.
Often presents in nasal cavity or nasopharynx

Genetics
Germline TCR
EBV present as closed episomal DNA

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16
Q

Extranodal NK/T Cell Lymphoma, Nasal Type- Immunophenotype

A 
Immunophenotype
CD2, CD56 positive
CD3, CD4, CD5, CD8, TCR negative
Cytotoxic granules positive
EBV positive
LMP-1
EBER
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17
Q

Enteropathy Associated TCL- Morphology

A

Type I
Usually presents as multiple ulcerating masses in the jejunum or ileum
Cells invade mucosa and range in size from small to large
Background mixed inflammatory infiltrate
Associated enteropathy in adjacent mucosa

Type II
Usually sporadic
Monomorphic

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18
Q

Enteropathy Associated TCL- Immunophenotype

A 
Type I	
CD3, CD7, *CD103 positive*
CD5, CD4 negative
CD8, CD56 variable
TCR a/b
Type II	
CD3, CD7, *CD103 positive*
CD5, CD4 negative
CD8, CD56
TCR g/d
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19
Q

Enteropathy Associated TCL- Genetics

A

Genetics
Clonal TCR rearrangement
HLA-DQA10501, DQB201 genotype

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20
Q

Hepatosplenic TCL- Morphology

A

Sinusoidal infiltrate of lymphoid cells involving bone marrow, liver, spleen
Cells intermediate in size/scanty cytoplasm
Nuclei oval to irregular with condensed chromatin and +/- small nucleoli

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21
Q

Hepatosplenic TCL- Immunophenotype

A

CD3, CD7, TIA-1, TCR-g/d positive
CD4, CD8, CD5 negative
Perforin and granzyme B usually negative (even though TIA-1 positive)

22
Q

Hepatosplenic TCL- Genetics

A

Isochromosome 7q

Clonal TCR

23
Q

Subcutaneous panniculitis like TCL- Morphology and Genetics

A

Morphology
Subcutaneous diffuse infiltrate
Epidermis and dermis typically spared
Cells rim fat cells
Cells small to large with moderate amount of cytoplasm
Nuclei oval to irregular with inconspicuous nucleoli

Genetics
Clonal TCR

24
Q

Subcutaneous panniculitis like TCL- Immunophenotype

A

CD3, CD8, TIA-1 positive
CD56-/+
TCR-a/b
EBV negative

25
Q

MF Morphology and Genetics

A

Epidermotropic infiltrate of small to intermediate size lymphs
Cells scanty cytoplasms, cerebriform nuclear contours and condensed chromatin
Pautrier microabscesses are characteristic
May involve lymph node ranging from small clusters atypical cells to effacement of architecture

TCR

26
Q

MF Variants

A

Pagetoid reticulosis
Folliculotropic MF
Granulomatous slack skin

27
Q

MF Immunophenotype

A

CD2, CD3, CD4 (most) or CD8 (rare), CD5 positive

CD7 negative

28
Q

MF Staging

A

Stage I: limited to skin; no LN involvement
Stage II: N1-N2 LN involvement or tumors
Stage III: erythroderma, no or N1-N2 LN involvement, low
circulating SS cells
Stage IV: high circulating SS cells or N3 LN involvement

29
Q

Sezary Syndrome

A

Erythroderma + LAD + Circulating SS cells
SS cell count:
>1000/uL
CD4:CD8 >10:1
loss of CD7, CD26 or other T cell antigens

30
Q

Primary Cutaneous CD30+ T LPD

A 
Primary cutaneous anaplastic large cell lymphoma (C-ALCL)
Lymphomatoid papulosis (LyP)
31
Q

Primary C-ALCL Morphology

A

Diffuse infiltrate typically involving dermis and subcutaneous tissue, similar to systemic ALCL

32
Q

Primary C-ALCL Immunophenotype

A

CD4, CD30 positive
CD2, CD3, CD5 variable
EMA, ALK-1 negative

33
Q

Primary C-ALCL Genetics

A

Clonal TCR
No t(2;5) or other translocations involving ALK
May have IRF4/DUSP22 translocation

34
Q

LyP Clinical

A

Crops of spontaneously resolving papules

35
Q

LyP Mophology

A

Wedge shaped polymorphic dermal infiltrate with mixture of atypical lymphocytes and acute and chronic inflammatory cells
May have RS-like cells (Type A lesion) or MF-like (Type B lesion) or ALCL-like (Type C) morphology

36
Q

LyP Immunophenotype

A

Atypical T cell phenotype, CD4 positive
Pan-T-cell markers variably expressed
CD30 positive cells in type A/C lesions

37
Q

LyP Genetics

A

Clonal TCR rearrangement in approximately 1/2 of cases

38
Q

Primary Cutaneous TCL, Rare Subtypes

A

Primary cutaneous gamma/delta TCL
Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic TCL
Primary cutaneous CD4 positive small/medium TCL

39
Q

Primary Cutaneous Gamma/Delta TCL Morphology and Genetics

A

Epidermis, dermis, and subcutis may all be involved
Cells rim fat cells if involving subcutis
Cells are small to large with moderate amount of cytoplasm
Nuclei oval to irregular; +/- conspicuous nucleoli

Clonal TCR

40
Q

Primary Cutaneous Gamma/Delta TCL Immunophenotype

A 
CD3+,CD4-CD8+ or CD3+,CD4+,CD8-, TIA-1+
CD5 negative
CD56 -/+
TCR-g/d
EBV negative
41
Q

PTCL, NOS Morphology

A

Diffuse infiltrate of cells- often a mixture of small, intermediate and large cells with varying proportions

Variants:
T zone variant
Follicular variant
Lymphoepithelioid cell variant (Lennert lymphoma)

42
Q

PTCL, NOS Morphology

A

CD4 positive
Variable expression of CD2, CD3, CD5, CD7
Usually lack cytotoxic granule associated proteins

43
Q

PTCL, NOS Genetics

A

Follicular variant with t(5;9)(q33;q22) involving ITK and SYK genes
Clonal TCR

44
Q

AITL Morphology

A

Partially or completely effaced lymph node with regressed lymphoid follicles
Infiltrating cells are polymorphous small to intermediate sized cells including clear cell immunoblasts
Admixed inflammatory cells in background and increased follicular dendritic cells
Arborizing high endothelial venules are increased

45
Q

AITL Immunophenotype

A

CD3, CD4 positive

T follicular helper markers frequently positive:
CD10, BCL6, PD1 (CD279), SAP (SH2D1A), IL21, ICOS, CXCR5, and CXCL13

Increased CD21 positive dendritic cells

46
Q

AITL Genetics

A

Frequent TET2 mutations
EBV sequences may be detected
Clonal TCR

47
Q

ALCL, ALK + Morphology

A

Large pleomorphic cells with abundant cytoplasm and horseshoe/kidney shaped nuclei (Hallmark cells); involve LN in sinusoidal pattern

Variants:

1) Common variant (70%)- majority of cells Hallmark cells
2) Lymphohistiocytic variant (10%)- large number of admixed histiocytes
3) Small cell variant (5-10%)- small to medium sized cells, Hallmark cells present

48
Q

ALCL, ALK + Immunophenotype

A

CD30, ALK1, EMA, clusterin, cytotoxic associated antigens (TIA-1, perforin, granzyme B) positive
Pan-T cell antigens show variable expression, most commonly CD43, CD2, and CD4 positive

49
Q

ALCL, ALK + Genetics

A

t(2;5) NPM-ALK or variants

Clonal TCR

50
Q

ALCL, ALK – Morphology

A

Large pleomorphic cells similar to those in ALCL, ALK +

51
Q

ALCL, ALK – Immunophenotype

A

CD30, EMA, cytotoxic associated antigens (TIA-1, perforin, granzyme B) positive
Pan-T cell antigens show variable expression, most commonly CD43, CD2, and CD4 positive
ALK negative
Rarely PAX5 positive due to gene amplification

Hematopathology (15 decks)

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