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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
MF Morphology and Genetics
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
MF Variants
Pagetoid reticulosis Folliculotropic MF Granulomatous slack skin
27
MF Immunophenotype
CD2, CD3, CD4 (most) or CD8 (rare), CD5 positive | CD7 negative
28
MF Staging
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
Sezary Syndrome
Erythroderma + LAD + Circulating SS cells SS cell count: >1000/uL CD4:CD8 >10:1 loss of CD7, CD26 or other T cell antigens
30
Primary Cutaneous CD30+ T LPD
``` Primary cutaneous anaplastic large cell lymphoma (C-ALCL) Lymphomatoid papulosis (LyP) ```
31
Primary C-ALCL Morphology
Diffuse infiltrate typically involving dermis and subcutaneous tissue, similar to systemic ALCL
32
Primary C-ALCL Immunophenotype
CD4, CD30 positive CD2, CD3, CD5 variable EMA, ALK-1 negative
33
Primary C-ALCL Genetics
Clonal TCR **No t(2;5) or other translocations involving ALK** May have IRF4/DUSP22 translocation
34
LyP Clinical
Crops of spontaneously resolving papules
35
LyP Mophology
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
LyP Immunophenotype
Atypical T cell phenotype, CD4 positive Pan-T-cell markers variably expressed CD30 positive cells in type A/C lesions
37
LyP Genetics
Clonal TCR rearrangement in approximately 1/2 of cases
38
Primary Cutaneous TCL, Rare Subtypes
Primary cutaneous gamma/delta TCL Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic TCL Primary cutaneous CD4 positive small/medium TCL
39
Primary Cutaneous Gamma/Delta TCL Morphology and Genetics
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
Primary Cutaneous Gamma/Delta TCL Immunophenotype
``` CD3+,CD4-CD8+ or CD3+,CD4+,CD8-, TIA-1+ CD5 negative CD56 -/+ TCR-g/d EBV negative ```
41
PTCL, NOS Morphology
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
PTCL, NOS Morphology
CD4 positive Variable expression of CD2, CD3, CD5, CD7 Usually lack cytotoxic granule associated proteins
43
PTCL, NOS Genetics
Follicular variant with t(5;9)(q33;q22) involving ITK and SYK genes Clonal TCR
44
AITL Morphology
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
AITL Immunophenotype
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
AITL Genetics
Frequent TET2 mutations EBV sequences may be detected Clonal TCR
47
ALCL, ALK + Morphology
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
ALCL, ALK + Immunophenotype
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
ALCL, ALK + Genetics
t(2;5) NPM-ALK or variants | Clonal TCR
50
ALCL, ALK -- Morphology
Large pleomorphic cells similar to those in ALCL, ALK +
51
ALCL, ALK -- Immunophenotype
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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