NK-lymphoblastic leukemia/lymphoma Flashcards

1
Q

What is the issue with this

disease entity ?

A
  • very difficult entity to define
  • many of the cases in the literature previously that were CD56 positive have been found to be BPDCN
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2
Q

What is true about NK cell

progenitors when they develop ?

A
  • they express no specific markers OR they express markers that overlap with T-ALL
    • CD7, CD2
    • even CD5 can be positive
    • cCD3 epsilon
  • CD16
    • more mature marker of NK cells
    • rarel ever expressed in NK cell leukemia

IMP: difficult to distinguish between T-ALL and NK-cell tumors

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3
Q

What are other relatively specific markers

for NK cells ?

A
  • relatively specific and expressed on NK cell progenitors
    • CD94
    • CD161
    • unfortunately these are uncommonly tested
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4
Q

In what circumstance can a diagnosis of NK

lymphoblastic leukemia/lymphoma be considered ?

A
  • as of now this is a provisional entity
  • can consider this diagnosis IF:
    • cells express CD56
    • immature T associated markers: CD7, CD2, cCD3
    • lack of B and myeloid antigens
    • TR and IG genes are in the germline configuration
    • BPDCN has been excluded
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