Mast cell activation syndrome as a significant comorbidity in sickle cell disease

Lawrence B. Afrin

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


Some sickle cell anemia (SCA) patients suffer significantly worse phenotypes than others. Causes of such disparities are incompletely understood. Comorbid chronic inflammation likely is a factor. Recently, mast cell (MC) activation (creating an inflammatory state) was found to be a significant factor in sickle pathobiology and pain in a murine SCA model. Also, a new realm of relatively noncytoproliferativeMC disease termedMC activation syndrome (MCAS) has been identified recently. MCAS has not previously been described in SCA. Some SCA patients experience pain patterns and other morbidities more congruent with MCAS than traditional SCA pathobiology (eg, vasoocclusion). Presented here are 32 poorphenotype SCA patients who met MCAS diagnostic criteria; all improved with MCAS-targeted therapy. As hydroxyurea benefits some MCAS patients (particularly SCA-like pain), its benefit in SCA may be partly attributable to treatment of unrecognized MCAS. Further study will better characterize MCAS in SCA and identify optimal therapy.

Original languageEnglish (US)
Pages (from-to)460-464
Number of pages5
JournalAmerican Journal of the Medical Sciences
Issue number6
StatePublished - 2014

Bibliographical note

Funding Information:
Supported by the Linda Summerall Mast Cell Research Fund and the Kathy Klein Mast Cell Research Fund at the University of Minnesota Foundation, University of Minnesota, Minneapolis, MN.

Publisher Copyright:
Copyright © by the Southern Society for Clinical Investigation.


  • Hydroxyurea
  • Kit mutations
  • Mast cell activation syndrome
  • Pain
  • Sickle cell anemia


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