Difference between revisions of "Mastocytosis"

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#redirect [[Non-malignant_skin_disease#Mastocytosis]]
{{ 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:Non-malignant skin disease]]

Latest revision as of 03:19, 20 March 2018

Mastocytosis
Diagnosis in short

Mastocytosis. H&E stain.
LM 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)
LM DDx intradermal nevus
Stains Giemsa stain +ve
IHC CD117 +ve, tryptase +ve
Site skin
Prevalence not very common
Prognosis dependent on underlying cause
Treatment dependent on underlying cause

Mastocytosis is the abundance of mast cells. It can be due to a number of causes.

General

Classification:[1]

  1. Cutaneous (only) - usually children.
    • Urticaria pigmentosa.
    • Others.
  2. Systemic - usually adults.
    • Indolent subvariant.
    • Aggressive subvariant.
    • Leukemic subvariant.

Microscopic

Features:[2]

  • 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:

Images

www:

  • Mastocytosis - intermed. mag. (WC)

  • Mastocytosis - high mag. (WC)

  • Mastocytosis - very high mag. (WC)

  • Mastocytosis - very high mag. (WC)

Stains

IHC

  • CD117 +ve.
  • Tryptase +ve.[3]

See also

References

  1. ↑ Arock, M.; Valent, P. (Aug 2010). "Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives.". Expert Rev Hematol 3 (4): 497-516. doi:10.1586/ehm.10.42. PMID 21083038.
  2. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1185. ISBN 978-1416031215.
  3. ↑ Rudzki, Z.; Sotlar, K.; Kudela, A.; Starzak-Gwóźdź, J.; Horny, HP. (2011). "Systemic mastocytosis (SM) and associated malignant bone marrow histiocytosis - a hitherto undescribed form of SM-AHNMD.". Pol J Pathol 62 (2): 101-4. PMID 21866466.
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