Systemic Mastocytosis

systemic mastocytosis

systemic mastocytosis and maculopapular cutaneous lesions (urticaria pigmentosa)

Systemic mastocytosis (SM) is a form of mastocytosis in which mast cells accumulate in internal tissues and organs such as the liver, spleen, bone marrow, and small intestines. It is typically diagnosed in adults. Signs and symptoms vary based on which parts of the body are affected.

The World Health Organization (WHO) classification of mastocytosis includes the following:

  • Cutaneous mastocytosis – Urticaria pigmentosa, maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis, mastocytoma of skin.
  • Indolent systemic mastocytosis.
  • Systemic mastocytosis with an associated (clonal) hematologic non–mast cell lineage disease.
  • Aggressive systemic mastocytosis.
  • Mast cell leukemia.
  • Mast cell sarcoma.
  • Extracutaneous mastocytoma.
References:

Jacquiline Habashy, Dirk M Elston. Mastocytosis: Background, Pathophysiology, Etiology. https://emedicine.medscape.com/article/1057932-overview

Patnaik MM, Rindos M, Kouides PA, Tefferi A, Pardanani A. Systemic mastocytosis: a concise clinical and laboratory review. Arch Pathol Lab Med. 2007 May. 131(5):784-91.

Valent P. Diagnostic evaluation and classification of mastocytosis. Immunol Allergy Clin North Am. 2006 Aug. 26(3):515-34.

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