T Cell

Question 1

Common clinical presentations

Answer 1

B symptoms
Marrow involvement(70%)
ASV stage 
Ldh 
Anaemia
Hyperoes 
Hypergamma

Question 2

AITCL - commonly the masqueraded of what

Answer 2

Immune activator- , high ear, elevated autoimmune serology
Circulating immune complexes or cold agg’s
10% clonal plasmacytosis
Warm AIHA
Oes +/- infectious aetiology

Question 3

Cell of origin for AITL

Answer 3

Follicular t helper cell

Question 4

Normal role of T foll helper cell

Answer 4

T f H cells live on outside of germinal centre and chaperone B cell after antigenic stimulation to becoming mem cells or plasma cells

Question 5

Phenotype of t fh cells

Answer 5

Cd 3 Cd4 and cd 10 +ve
The T cell receptor is alpha-beta with aberrant loss of CD5 and/or CD 7
CD30 seen in 20% cases
Near uniform expression of cytoplasmic cxcl13

Question 6

Bone marrow aitl

Answer 6

Hyper cellular
Lymphocyte infiltrate w no distinct pattern
Reactive cellular component may be present
Oesinophilia inc plasma cells

Question 7

Flow panel for AITL

Answer 7

Cd2-3-4-5-7-8-10 are +Ve
Pd1, cxcl13, ICOS- markers of t foll help cells, often +Ve
EBER ish- +Ve in B cells
Cd246(alk)-20-79a all neg
Loss of expression of pan T cell markers not uncommon- usually predominance cd 4 over cd 8

Question 8

Helpful immunophenotypic features to help establish clonality in T cells

Answer 8

T-cell subset antigen restriction,
anomalous T-cell subset antigen expression,
deletion or diminution of one of the pan T-cell antigens,
a precursor T-cell phenotype
expression of additional markers (e.g., CD30, CD20, major myeloid antigens, and TCRγδ)

Question 9

The most common all fusion partner in Anaplastic

Answer 9

Npm t 2:5

Question 10

Gene involved in alk-Ve alc

Answer 10

Dusp22 and has a few diff fusion partners

Question 11

T PLL immunophenotype

Answer 11

Strong cd7(which most others aren't)
cCD3 rather than surface 
Cd2/5/7+ve 
60% cd4+ but can have cd 8 and double expression 
TCR a/b
Cd 54 +( hence campath)

Question 12

T LGL clinical

Answer 12

Anaemia
Profound neutropaenia
Plts not affected
Splenomegaly
Immune complex formation, autoantibodies hypergammaglobulinaemia
If cd 4+Ve - often associated with underlying malignancy

Question 13

Bone marrow in lgl

Answer 13

Normocellular in 50%
Left shifted granulocytes
Mild to moderate reticulin fibrosis

Question 14

Flow t lgl

Answer 14

Diminished or lost e/o cd5 and or cd7
Cd 57 and CD 16 expressed in over 80%
Express tia 1, granzyme b and M

Question 15

Abnormal flow in T cell disorders

Answer 15

loss or markedly dim expression of CD45;
complete loss of one or more pan-T antigens;
diminished expression of more than two pan-T antigens in conjunction with altered light scatter properties;
CD4/CD8 dual-positive or dual-negative expression

Question 16

Presentation ATL

Answer 16

55 years
lymphadenopathy (72%),
skin lesions (53%),
hepatomegalysplenomegaly (25%), hypercalcemia (28%).6
Cellular immunosuppression is common
significant minority of patients may have concurrent strongyloides infection

Question 17

Flow ATL

Answer 17

CD3, CD4, CD25, and CD52

Question 18

Differentiating tpll, ss, mf ATLL

Answer 18

that T-PLL typically shows no antigen loss and expresses uniform CD26; SS and MF frequently show loss of CD7 and CD26 but absence of CD25; ATLL shows loss of CD7 and loss of CD26 but is consistently CD25 +

Question 19

Cell of origin in ATL

L

Answer 19

CD4 + CD25 + FOXP3 + regulatory T cells

Question 20

Infectious association ATLL

Answer 20

Htlv1

Question 21

Flow for Sezary

Answer 21

Loss of expression of CD7 and CD26 is typical of Sézary cells but it must be remembered that CD7 loss or weak expression may also be seen in peripheral blood T cells from patients with benign dermatoses. Loss of CD26 expression may be a more robust marker of neoplasia

Question 22

CG t pll

Answer 22

Usually complex

Often inv 14 or tx:14

Question 23

Hepatosplenic cg

Answer 23

Isochromosome 7 q where q arm is duplicated and placed over where the p arm used to be so get three copies of q

Question 24

Peripheral blood in TLGL

Answer 24

Neutro

Inc numbers of lGL- count > 2 x 10(9)

Question 25

nodal T-cell lymphomas with T-follicular helper (TFH) phenotype

Answer 25

angioimmunoblastic T-cell lymphoma (AITL),
follicular T-cell lymphoma and
nodal peripheral T-cell lymphoma (PTCL) with a TFHphenotype