title: Mast cell activation syndrome as a significant comorbidity in sickle cell disease.
abstract: 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 noncytoproliferative MC disease termed MC 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 poor-phenotype 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.
authors.affiliation: Division of Hematology, Oncology & Transplantation, University of Minnesota, Minneapolis, Minnesota.
authors.forename: Lawrence B
authors.initials: LB
authors.lastname: Afrin
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D000986:Antisickling Agents / Q000627:therapeutic use*; D002364:Caseins / Q000097:blood; D053379:Chromogranin A / Q000097:blood; D015331:Cohort Studies; D005260:Female; D006493:Heparin / Q000097:blood; D006632:Histamine / Q000097:blood; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D007249:Inflammation; D008297:Male; D008415:Mastocytosis / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D008761:Methylhistamines / Q000652:urine; D008875:Middle Aged; D010641:Phenotype; D015230:Prostaglandin D2 / Q000097:blood / Q000652:urine; D011492:Protein Hydrolysates / Q000097:blood; D013577:Syndrome; D055815:Young Adult
chemical_list: D000986:Antisickling Agents; D002364:Caseins; D053379:Chromogranin A; D008761:Methylhistamines; D011492:Protein Hydrolysates; C026375:trypticase-soy broth; D006632:Histamine; D006493:Heparin; D015230:Prostaglandin D2; D006918:Hydroxyurea; C048140:N-methylhistamine