Acute Myeloid Leukemia with Occult Systemic Mastocytosis or Atypical Mast Cells Post-induction

Systemic mastocytosis (SM) is a state of disease that is related to the clonal, neoplastic proliferation of mast cells. Patients who present with SM-Acute Myeloid Leukemia (AML) often have the worst outcome. We present a case of an 18-year-old female who was diagnosed with AML (FLT3 (Fms like tyrosine kinase 3) and PML-RARA (promyelocytic leukemia-retinoic acid receptor alpha) translocation-negative) and after initial treatment with a standard induction regimen of cytarabine and daunorubicin (3+7 regimen), her bone marrow showed blast cells less than 5% and dense aggregates/sheets of atypical/immature mast cells with immunohistochemical stain CD117+ve and toluidine blue positive in mast cell aggregates. Mastocytosis is a clonal neoplastic proliferation of mast cells that accumulate in one or more organ system. Therefore, it is essential to diagnose systemic mastocytosis, particularly in patients of hematological neoplasms.