tryptasemia logoWhen you have a mystery illness, it's hard enough to get diagnosed with the recognizable disorders. It's important to remember: often, doctors only diagnose disorders they're familiar with.

Hereditary Alpha Tryptasemia Syndrome (HATS) is a relatively new disorder--first named in publications in 2015 and 2016. But there are hundreds of new disorders that are identified, and some of them are found to be duplicative. 

In the meantime, anyone who has been confirmed genetically to have one or more extra copies of TPSAB1, would be coded in the International Classification of Disease as follows:

D89.49, Other mast cell activation disorder.

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However, it is advantageous for HATS to be added to The International Classification of Diseases, Tenth Revision--more commonly known as ICD-10, as D89.44 Hereditary Alpha Tryptasemia.

To accomplish this, Drs. Jonathan Lyons and David Berglund (Translational Allergy and Immunopathology, NIAID, National Institutes of Health) were on the agenda of the ICD-10 Coordination and Maintenance Committee Meeting on September 8-9, 2020. The new code was requested by the following:

  • The University of Mississippi, Dr. Sarah Glover, Gastroenterology - Dr. Glover is both a HATS researcher and patient
  • The Mast Cell Diseases Society (formerly The Mastocytosis Society) - a nonprofit that sponsors research and supports patients with mast cell-related disorders
  • Gene by Gene, Inc. - accompany that performs specialized TPSAB1 genetic testing


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Source (also, see above under "References")

2020 September 8-9, Minutes of the International Classification of Diseases Committee Meeting.




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