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Mastocytosis

Abstract

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Synonyms of Mastocytosis

  • Systemic Mast Cell Disease
  • Systemic Mastocytosis

Disorder Subdivisions

  • Aggressive Systemic Mastocytosis
  • Cutaneous Mastocytosis
  • Indolent Systemic Mastocytosis
  • Mast Cell Leukemia
  • Mast Cell Sarcoma/Extracutaneous Mastocytoma
  • Mastocytosis with an Associated Hematological Disorder
  • uticaria pigmentosa

General Discussion

Mastocytosis is a rare disorder characterized by abnormal accumulations of mast cells in skin, bone marrow, and internal organs such as the liver, spleen and lymph nodes. Cases beginning during adulthood tend to involve the inner organs in addition to the skin whereas, during childhood, the condition is often marked by skin manifestations with minimal or no organ involvement. When there is evidence of bone marrow or internal organ involvement, the disease is referred to as "systemic mastocytosis".

Although the majority of cases follow an indolent course, some patients may have evidence of a blood disorder such as a myelodysplastic or myeloproliferative disorder at the time of diagnosis. The course and prognosis of mastocytosis in these patients are determined by this associated hematologic disorder. More aggressive forms of mastocytosis and mast cell leukemias are very rarely encountered.

Organizations related to Mastocytosis

NORD offers an online community for this rare disease. RareConnect was created by EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders) to provide a safe space where individuals and families affected by rare diseases can connect with each other, share vital experiences, and find helpful information and resources. You can view these international, rare disease communities at www.rareconnect.org.

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