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Difference between revisions of "Mastocytosis"
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# | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Mastocytosis_-_cropped_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Mastocytosis. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = mast cells (cells in the superficial/mid dermis that are: lymphocyte-like with more cytoplasm that is granular; cells may have spindled or stellate morphology); +/-eosinophils (common) | |||
| Subtypes = | |||
| LMDDx = [[intradermal nevus]] | |||
| Stains = [[Giemsa stain]] +ve | |||
| IHC = CD117 +ve, tryptase +ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[skin]] | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = not very common | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = dependent on underlying cause | |||
| Other = | |||
| ClinDDx = | |||
| Tx = dependent on underlying cause | |||
}} | |||
'''Mastocytosis''' is the abundance of [[mast cells]]. It can be due to a number of causes. | |||
==General== | |||
*Abundance of [[mast cell]]s. | |||
Classification:<ref name=pmid21083038>{{Cite journal | last1 = Arock | first1 = M. | last2 = Valent | first2 = P. | title = Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives. | journal = Expert Rev Hematol | volume = 3 | issue = 4 | pages = 497-516 | month = Aug | year = 2010 | doi = 10.1586/ehm.10.42 | PMID = 21083038 }}</ref> | |||
#Cutaneous (only) - usually children. | |||
#*Urticaria pigmentosa. | |||
#*Others. | |||
#Systemic - usually adults. | |||
#*Indolent subvariant. | |||
#*Aggressive subvariant. | |||
#*Leukemic subvariant. | |||
==Microscopic== | |||
Features:<ref name=Ref_PBoD8|1185>{{Ref PBoD8|1185}}</ref> | |||
*Cells in the superficial/mid dermis that are: | |||
**Lymphocyte-like with more cytoplasm that is granular. | |||
***Cells may have spindled or stellate morphology. | |||
***Tend to be more abundant around vessels. | |||
*+/-Eosinophils (common). | |||
*+/-Edema - often prominent; gives cells a white halo. | |||
Notes: | |||
*Lymphocyte versus mast cell: | |||
**Lymphocytes = round; mast cells = ovoid. | |||
DDx: | |||
*[[Intradermal nevus]]. | |||
*[[Pleomorphic undifferentiated sarcoma]], [[Langerhans cell histiocytosis]] and other [[pleomorphic tumours]] - for ''aggressive systemic mastocytosis''. | |||
===Images=== | |||
www: | |||
*[http://www.jameswpattersonmd.com/case_studies/index.cfm?CFID=387434 Mastocytosis - low res. (jameswpattersonmd.com)]. | |||
*[http://path.upmc.edu/cases/case366.html Mastocytosis - bone marrow - several images (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case409.html Systemic mastocytosis - several images (upmc.edu)]. | |||
<gallery> | |||
Image:Mastocytosis - intermed mag.jpg | Mastocytosis - intermed. mag. (WC) | |||
Image:Mastocytosis_-_high_mag.jpg | Mastocytosis - high mag. (WC) | |||
Image:Mastocytosis_-_very_high_mag.jpg | Mastocytosis - very high mag. (WC) | |||
Image:Mastocytosis_-_cropped_-_very_high_mag.jpg | Mastocytosis - very high mag. (WC) | |||
</gallery> | |||
==Stains== | |||
*[[Toluidine blue stain|Toluidine blue]] -- highlights the granules. | |||
*[[Giemsa stain]] +ve | |||
==IHC== | |||
*CD117 +ve. | |||
*Tryptase +ve.<ref name=pmid21866466>{{Cite journal | last1 = Rudzki | first1 = Z. | last2 = Sotlar | first2 = K. | last3 = Kudela | first3 = A. | last4 = Starzak-Gwóźdź | first4 = J. | last5 = Horny | first5 = HP. | title = Systemic mastocytosis (SM) and associated malignant bone marrow histiocytosis - a hitherto undescribed form of SM-AHNMD. | journal = Pol J Pathol | volume = 62 | issue = 2 | pages = 101-4 | month = | year = 2011 | doi = | PMID = 21866466 }} | |||
</ref> | |||
==See also== | |||
*[[Non-malignant skin disease]]. | |||
*[[Mast cell]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Non-malignant skin disease]] | |||