mature T cell

Question 1

PTCL-NOS

Answer 1

gen lymphaden
eosinophilia
pruritis, fever, weightloss

Question 2

anaplastic large cell lymphoma

Answer 2

ALK gene 
- tyrosine kinase 
horseshoe/wreath nuclei
sinusoidal pattern 
kids have good prognosis

T cell or null cell
no B cell Ag
CD30, EMA, ALK

Question 3

adult T-cell leukemia/lymphoma

Answer 3

HTLV1 – infects cd4 t cell
Caribbean basin, Japan, W. Africa

Leukemic: rapidly progressive, die w/in 1 yr despite chemo

localized to skin: much less aggressive

CD4+/CD7-
HTLV-1 +

Question 4

Large granular lymphocytic leukemia

Answer 4

T cell (CD3+)/ NK cell (CD3-)

STAT3 mutation (30%)

neutropenia w/ dec myeloid maturation in BM
anemia
Rheumatologic disease assoc (autoimmune disease) - Felty syndrome – Rh arthritis, splenomegaly, neutropenia – LGL as underlying cause

T cell LGL = indolent
NK cell LGL = aggressive