Mastocytosis

From Libre Pathology
Jump to: navigation, search
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:

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.