A Brief History Of Mast Cell Disease

Aside from knowing what to eat, which doctors to see, which medicines to take and what symptoms are commonly seen in mast cell disorders of all types, have you ever wondered about the history of this little immune cell that (for many of us) is wreaking havoc in our lives?

Here is a brief history of the mast cell, mastocytosis and mast cell activation disease.

Much of the following is taken from information provided in the presentation video entitled: Mast Cell Activation Disease: Current Concepts by Dr. Lawrence Afrin (see below).

History of the mast cell:

Mast cells were first described by Paul Ehrlich in his 1878 doctoral thesis on the basis of their unique staining characteristics and large granules. These granules also led him to the incorrect belief that they existed to nourish the surrounding tissue, so he named them Mastzellen (from German Mast, meaning “fattening”, as of animals). They are now considered to be part of the immune system.

Mast cells are very similar to basophil granulocytes (a class of white blood cells) in blood. Both are granulated cells that contain histamine and heparin, an anticoagulant. Both cells also release histamine.

History of Mastocytosis:

  • 1869 – Urticaria Pigmentosa first described
  • 1877 – First description of the mast cell or mastzelle (Paul Ehrlich)
  • 1887 – UP linked with mast cells
  • 1933 – Mast cells linked with internal diseases
  • 1939 – Heparin in mast cells (first mediator) identified
  • 1949 – Definitive linkage with systemic disease
  • 1953 – Histamine in mast cells identified
  • 1987 – Tryptase in mast cells identified
  • 1988 – Classification system of mastocytosis introduced
  • 1995 – KIT activation mutation D816V identified
  • 1998 – Unique flow cytometric signature found
  • 2001 – WHO (World Health Org) classification & imatinib (Gleevec) approved for mastocytosis (KIT neg patients)

History of Mast Cell Activation Syndrome:

  • 1991 – published hypothesis that MCAS should be recognized
  • 2007 – 1st case reports of MCAS (some with KIT-D816v)
  • 2007 – 1st study showing other KIT mutations in most MCAS (Bonn Study)
  • 2008 – Non-KIT mast cell regulatory gene mutations found in SM
  • 2010 – 2nd study showing KIT mutations in most MCAS (Bonn). Also showed KIT-D816V rare in MCAS & few mutations in controls.
  • 2010 – 1st proposal for diagnostic criteria of MCAD
  • 2011 – alternative proposal for MCAS dx suggested
  • 2012 – revised proposal for MCAS dx proposed (still problems)
  • 2016 – revised WHO diagnostic criteria for SM (systemic mastocytosis)

You can watch the entire presentation by Dr. Afrin here:

 

Please visit The Mastocytosis Society for more information.

 

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