Mast Cell Activation Syndrome as a Significant Comorbidity in Sickle Cell Disease

摘要: 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 (MC) activation (creating an inflammatory state) was found to be significant factor in pathobiology and pain murine SCA model. Also, new realm relatively noncytoproliferative MC disease termed syndrome (MCAS) has been identified recently. MCAS not previously described SCA. experience patterns other morbidities more congruent with traditional (eg, vasoocclusion). Presented here 32 poor-phenotype who met diagnostic criteria; all improved MCAS-targeted therapy. As hydroxyurea benefits some (particularly SCA-like pain), its benefit may partly attributable treatment unrecognized MCAS. Further study will better characterize identify optimal