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LPD > T and NK cell LPD > Flashcards

T and NK cell LPD Flashcards

1
Q

T Follicular Helper immunophenotype

A

CD10, BCL6, CXCL13, CXCR5, ICOS, PD1, SAP

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2
Q

Clinical features of AITCL

A

Patients generally present with advanced disease

  1. Generalised lymphadenopathy
  2. Hepatosplenomegaly
  3. Skin rash +/- pruritus
  4. Ascites, pleural effusion, arthritis
  5. Often associated with autoimmune conditions
  6. Immunodeficiency
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3
Q

Laboratory features of AITCL

A
  1. Hypergammaglobulinaemia
  2. RF and anti-smooth muscle antibodies
  3. Cold agglutinins with haemolytic anaemia
  4. Circulating immune complexes
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4
Q

Immunophenotype of T-PLL

A

Positive T cell markers: CD2, CD5, CD3 and CD7 (CD3 can be weak)
CD52 is usually strongly expressed and can be a target of therapy
60% CD4+/CD8-
25% CD4+/CD8+ (dual positivity is not really seen in any other mature T cell neoplasm, ddx T-ALL)
15% CD4-/CD8+
Negative: CD1a, TdT

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5
Q

Features of T-PLL (disease of 52)

A 
Aggressive T-cell leukaemia
Affects older adults (52 year olds)
PB lymphocyte count > 100 (520)
CD2+, CD5+, CD52+
5 cytogenetic abnormalities, 2 are very common
Inv(14) or t(14;14)
Trisomy 8
Deletions at 12p and amplification of 5p
TP53
Median survival 5 months to 2 years

Anaemia and thrombocytopenia
Lymphadenopathy, hepatosplenomegaly
Skin infiltration in 20% (blebs = skin lumps)

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6
Q

What are the cytogenetics abnormalities in T-PLL?

A
  1. Inversions of chromosome 14
    - Inv(14) in 80%, t(14;14) in 10%
  2. Abnormalities of chromosome 8
    - idic(8), t(8;8) and trisomy 8q are seen in 80%
  3. Deletions at 12p13 and amplification of 5p are also common
  4. TP53 deletions
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7
Q

Clinical and laboratory features of aggressive NK cell leukaemia

A
  • Patients usually present with fevers, weight loss, fatigue
  • Pancytopenia with leukaemic cells
  • Hepatosplenomegaly
  • Effusions are common
  • High LDH
  • May have coagulopathy, haemophagocytic syndrome or multiorgan failure
  • Median survival < 2months
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8
Q

Is aggressive NK cell leukaemia EBV driven?

A

Yes - 80-100% association with EBV. Seen in adults, median age is 40 years.
N.B. Chronic lymphoproliferative disorder of NK cells is EBV negative.

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9
Q

Immunophenotype of extranodal NK/T cell lymphoma

A

CD2+, sCD3- (but CD3ε+), CD56+, usually CD16-, CD4- and CD8-
Cytotoxic molecules positive
(EBV positive)

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10
Q

Immunophenotype of Adult T-cell leukaemia/lymphoma

A

T cell antigens: CD2+, CD3+, CD5+, usually CD4+
CD7-
CD25 is strongly expressed in nearly all cases
CD30 may be positive

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11
Q

Clinical features of ATLL

A

Acute - leukaemic presentation with high WBCs, high LDH, hypercalcaemia, hepatosplenomegaly, lytic bone lesions, rash and BM infiltration.
Lymphomatous - LNs but no PB, otherwise similar to acute form.
Chronic - mildly increased lymphocytes, mildly raised LDH. May have hepatosplenomegaly. Ca2+ normal.
Smouldering - leukaemic cells >5% but WBC count normal. No systemic symptoms.

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12
Q

Features of Sezary Syndrome (triad + additional)

A
  1. Erythroderma
  2. Generalised lymphadenopathy
  3. Leukaemia - abnormal clonal cerebriform T cells circulating
    Plus one of the following additional criteria:
    Sezary count >1000/uL
    CD4:CD8 >10:1
    Loss of T cell antigens e.g. CD7 and CD26
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13
Q

Immunophenotype of Sezary Syndrome

A

CD3+/CD4+/CD8-
CD7- and CD26-
PD1 +

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14
Q

Define T-LGL

A

Persistent (>6 months) increase in LGLs in the peripheral blood
Usually 2-20

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15
Q

What are reactive causes of increased T-LGLs?

A
  1. Infection
  2. G-CSF
  3. Immune reconstitution following SCT and chemotherapy
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16
Q

What are the clinical features of T-LGL?

A
  1. Usually affects middle aged to older adults
  2. Indolent
  3. Symptomatic from cytopenias - neutropenia is common, then anaemia. Thrombocytopenia is rare.
  4. Splenomegaly
  5. Associated with RA
  6. Hypergammaglobulinaemia and immune complexes
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17
Q

Describe morphology in T-LGL

- PB and BM

A
  • Intermediate to large lymphocytes, abundant cytoplasm and fine or coarse azurophilic granules
  • BM hypocellular/normocellular /mildly hypercellular
  • Intrasinusoidal +/- interstitial infiltrates
  • BM fibrosis common
  • Granulocytic hypoplasia
  • Very common to find reactive lymphoid aggregates (CD4+ lymphocytes rather than CD8+)
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18
Q

What is the immunophenotype of T-LGL?

A

CD3+, CD8+, CD16+, CD57+ (CD56-, dim CD5 and dim CD7) with alpha/beta TCR gene rearrangement
TIA1, granzyme

Rare variants:

  1. alpha/beta with CD4+
  2. gamma/delta type rare and may be CD8+ or CD4-/CD8-
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19
Q

Definition of chronic lymphoproliferative disorder of NK cells

A

Rare and heterogenous disorder characterised by persistent (>6 months) increase in NK cells in the peripheral blood (>2)

20
Q

Morphology of NK-LGL

A
  • Intermediate to large lymphocytes, abundant cytoplasm and fine or coarse azurophilic granules
  • Intrasinusoidal +/- interstitial infiltrates
21
Q

Immunophenotype of NK-LGL

A

sCD3-, CD8+, CD16+, CD56+(dim) and cytotoxic markers

Often loss of CD2, CD5 and/or CD57

22
Q

Morphology of aggressive NK cell leukaemia

A

Variable - ranging from normal appearing LGLs through to cells with atypical nuclei, open chromatin and nucleoli
BM - interstitial or focal infiltrate +/- haemophagocytosis

23
Q

Immunophenotype of NK cell leukaemia?

A

CD2+, sCD3-, CD5-, CD16+, CD56+

24
Q

Definition of ATLL

A

Aggressive lymphoma/leukaemia caused by HTLV1 infection.
HTLV1 infections occur in childhood but disease has long latency and presents in adulthood
2.5% of carriers develop ATLL
Flowery lymphocytes

25
Q

Prognosis and predictive factors in ATLL

A

Acute and lymphomatous form 2weeks - 1 year
Chronic and smouldering forms progress to acute phase in 25%
Death from opportunistic infections is common e.g. PJP and strongyloides

26
Q

Definition of hepatosplenic T cell lymphoma

A

Aggressive extranodal T cell lymphoma with proliferation of cytotoxic T cells that are usually the gamma/delta TCR type

27
Q

Clinical features of hepatosplenic T cell lymphoma

A

Young adults (median age 35 years)
20% of cases arise in the setting of chronic immunosuppression e.g. infliximab + azathioprine in IBD patients.
Hepatosplenomegaly without lymphadenopathy
Marked thrombocytopenia +/- anaemia
BM almost always involved

28
Q

Morphology of hepatosplenic T cell lymphoma

A

Monotonous medium sized lymphocytes with a pale rim of cytoplasm and loosely condensed chromatin
Prominent intrasinusoidal pattern in liver and BM

29
Q

Immunophenotype of hepatosplenic T cell lymphoma

A

CD3+, CD8+/-, CD56+/-, gamma/delta
CD4-, CD5-
DDx T-LGL (57+)

30
Q

Definition of Peripheral T cell lymphoma, NOS

A

Heterogenous group of nodal and extranodal T cell lymphoma that don’t fit in any other WHO category. Excludes those with a T follicular helper phenotype

31
Q

Clinical Features of Peripheral T cell lymphoma, NOS

A

Aggressive
Mostly seen in adults
B symptoms common
Usually nodal but can be extranodal, rarely leukaemic
Eosinophilia, pruritus and rarely HLH can occur

32
Q

Immunophenotype of PTCL

A

Frequent loss of T cell antigens
CD3+
CD4+>CD8+ but can get double positive and double negative
Can have CD56+ and cytotoxic markers positive
CD15 and/or CD30

33
Q

Morphology of PTCL, NOS

A

Diffuse infiltrate which may be monomorphic or polymorphic
Medium to large cells, may have RS like cells
Can have histiocyte rich form

34
Q

Definition of AITLC

A

Neoplasm of mature T cells with a T follicular helper cell phenotype
Always associated with EBV positive B cells but the neoplastic T cells are negative

35
Q

Morphology of AITCL (PB, BM)

A

Blood film - may see rouleaux or eosinophilia
BM - small to intermediate sized T cells surrounded by inflammatory cells e.g. eosinophils, plasma cells, histiocytes, reactive lymphocytes
Increased bone marrow vascularity
May have follicular architecture or this may be effaced

36
Q

Immunophenotype of AITCL

A

CD10, BCL6, PD1, CXCL13, CXCR5, SAP, ICOS
CD2+, CD3+, CD4+, CD5+
EBV positive cells = B cells

37
Q

Anaplastic large cell lymphoma - Definition

A

T cell lymphoma characterised by large and pleomorphic lymphoid cells with horse shoe shaped nuclei and abundant cytoplasm.

38
Q

Most common translocation involving ALK gene?

A

t(2;5) = ALK on chromosome 2, NPM1 on chromosome 5

But there are many partners and the partner doesn’t alter prognosis.

39
Q

Clinical features of ALCL

A

Children and young adults
ALK+ has a better prognosis
Advanced stage disease and B symptoms at presentation are common
LNs and extranodal
Less likely to have mediastinal disease compared to cHL
30% have BM involvement - need IHC to diagnose

40
Q

Name the 4 morphologic variants of ALCL

A
  1. Common
  2. Lymphohistiocytic
  3. Small cell
  4. Hodgkin like
    * *all variants have Hallmark cells in them**
41
Q

IHC and immunophenotype in ALCL

A

CD30+ (uniform)
EMA+ (100% of ALK+ but 50% of ALK-) EMA is negative in PTCL.
CD4+, CD2+, CD5+
CD3- in 75%
ALK+
PAX5- (differentiates it from cHL)
N.B. There can be a “null phenotype” but TCR gene rearrangement is clonal

42
Q

Breast lymphoma

A

90% B cell

10% T cell - systemic ALCL, breast implant associated ALCL

43
Q

What mutations are seen in ~1/3 of T and NK cell LGL

A

STAT3 mutations

44
Q

Immunophenotype of ATLL

A

CD3+, CD4+, CD2+, CD5+, CD25+
CD7-
+/- CD30

45
Q

Differential diagnosis of T cell leukaemias

A

T-PLL (CD4+/CD8+, CD7 strong, CD52+)
T-LGL (CD8+, CD16+, CD57+)
Sezary (CD7-, CD26-, PD1+)
ATLL (CD7-, CD25+)

LPD (3 decks)

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