mast cell Flashcards
classification of mast cell d/o
CUTANEOUS
Urticaria pigmentosa: discrete yellow brown maculular pap or nodular plaque lesion with DARIER sign (urticaria and ertyhema when stroking lesion)
Diffuse cutnaeous mastocytosis - diffuse yellow thickned skin with no discrete lesions usu <3yo
Mastocytoma: soltairy reddish brown lesion in 3 mo of life and resolves spontaeously
Telegiectasia macrularis eruptiva perstans: macular tenelg inc mast cell around ilated cap and venules and in adults
SYSTEMIC
Indolent systemic mastocytosis
Most common, slow progression and good prog
Smouldering (high mast cell burden >30% BM space are MC, tryptase>200ng/ml
Isolated BM mastocytosis: BM without skin involvement
Systemic mastocytosis with hematologic nonmast cell lineage disease
Aggressive systemic mastocytosis:
tissue infiltration causing hepatic fibrosis, portal htn, malabsorption or cytopenia
Mast cell leukiemia: rare>10% of immature in MC in blood or >20% in BM
Mast cell sarcoma
Extracutnaeous mastocytoma
diagnostic criteria for cutaneous and systemic mastocytosis
Diagnostic criteria (1 major and one minor OR 3 minor)
MAJOR: BM or extracutaneous organ wiht mutlifocal dense infiltrate of >15 mast cell agg
MINOR:
Bx more then 25% mast cell are spindle shpaned or atypical morph
Detection of ckit d816v (in BM or non skin organ)
Expression of CD2 and or CD25 on CD1117 mast cell
Total serum trptase >20ng/ml
poor prog for Mastocytoiss?
Poor prognosis
Male gender, no cutaneous sx, constitution sx, anemia or low plt, abn LFT, bilobed mast cell nuclei, low fat percent in BM, assoc heme malign
diagnostic criteria for MCAS
1) sx of mast cell activation
2) inc in mast cell with 20%+2ng/ml within 4 hours of anaphylaxis
3) respond to meds that reduce the mediatros
name four diff btwn mast cellT and mast cell TC
mast cell T only in mucosa dont have c5ra have only tryptase need t cells scrool granules
mast cell TC found many tissue have all the mediators t cell independt have c5ra WHORLEd apperaence
Preformed mast cell medaitors
produced
preformed - histamine, chymase, tryptased, TNF and heparin
released: PDG2, LTC4 LTB4 and PAF after arachoinioid acid pathway activation 1-0-30min
four ways mast cell activate
Ige crossi link Igg bind fcE r1 C3a and C5a TLR ligand and nerve growth factor `
how is PGE2 and PGD2 different
PGE2 is made in Eo, and is BLOCKING eo and Mast cells and broncholiates and blocks 5 LO
PGD2 nronchocnstict, made mast cells and vascodialte and inc vascular permaibility and gets Eo baso th2 ILC2 chemotaxis
ddx for mast cell disorders
primary mast cell
1) mastocytosis
2) monoclonal MCAS
secondary allergic, cIU Mast cell activation from other cancers phhysical urtcaira autoimmune urtcaria
idopathic is
MCAS
idiaopthic urticara anagpylaxis or angioedmea
POTS fibromyalgia pheo carcinoid parahtyroid
Eoe EOG GERD IBS vasoactive peptide secreting tumor HyperIGE ehadache depression angioedema sclerderoma chronic urdtcaria
name the gi med u use for ppl who have mast cell activation
disodium cromoglycate
also use h2 and h2 antag avoid triggers epi gleevec if dont have mutation dexa for bones
what do you check the uirine for when diagnosing mast cell activation
urine: 24 hour for methyl histamine pgd2 11 beta prostaglanding f2a level
