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Flashcards in Mastocytosis Deck (15)
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1

A mitogen that plays a big role in mast cell growth and migration

SCF

2

Neoplasms of mast cells usually present as...

benign cutaneous lesions in kids (the most common is “urticaria pigmentosa”, aka itchy pigmented skin lesions).

3

When they OCCASIONALLY spread beyond skin...

they release lots of histamine and other mediators, causing systemic symptoms (flushing, abdominal pain, tachycardia, hypotension) that can can really confuse the clinician.

4

most common site of systemic involvement

bone marrow
(but can also involve other organs...i.e. spleen, liver, lymph nodes)

5

Important diagnostic indicator

serum tryptase level

6

Fusion protein and tyrosine kinase involved...

FIP1L1-PDGFRB (4 partners identified)
Some respond to imatinib!!

7

Morphology (bone marrow)

Bland looking cells, round/spindle shaped

8

Percent associated w second hematologic malignancy

30%

9

Genetics

Either cKIT mutants OR PDGFRA activation (FIP1) translocation

10

Clinical Presentation

Variable and Confusing

11

Morphology (General)

Aggregates of bland looking cells
round/sphericle shaped
sometimes w/ eosinophilia

12

Immunophenotype

Tryptase
CD117 (cKIT)
CD25 usually positive

13

Prognosis

Highly variable
depends on complex subclassification scheme

14

Aggregates of mast cells can be subtle an bland looking, so...

Immunostains are key!

15

cKIR

SCF receptor!

CD117