Am J Leuk Res | Volume 6, Issue 1 | Case Report | Open Access

Pediatric Systemic Mastocytosis in Association with Clonal Non-Mast Cell Lineage Hematopoietic Neoplasm: A Case Report and Literature Review

Somanath Padhi¹, Sonali Mohapatra^2*, Nakul Tikare², Lakshmi R¹, Gargi Phukan¹

¹Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, India ²Department of Medical Oncology/Hematology, All India Institute of Medical Sciences, India

^*Correspondance to: Sonali Mohapatra 

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Abstract

Systemic mastocytosis associated with clonal non-mast cell lineage hematopoietic neoplasm (SMAHM) (usually myeloid) is sporadically reported to occur in both adults and elderly population. Occurrence of SM-AHM in pediatric population (≤ 14 years) is rarely reported with paucity of data pertaining to its biological behavior and therapeutic outcome. We described a case of SM in association with t (8; 21) (q22; q22) positive Acute Myeloid Leukemia (AML) in a young child with negative KIT D816V mutation with characteristic bone marrow morphologic and immunophenotypic features and unfavorable outcome following intensive chemotherapy. We also presented a comprehensive review of similar cases in pediatric population to highlight the morphologic, molecular, and biological behavior of such potentially unfavorable disease.

Keywords:

Mastocytosis; Acute myeloid leukemia; Cytogenetics; KIT; Prognosis

Citation:

Sonali M. Pediatric Systemic Mastocytosis in Association with Clonal Non-Mast Cell Lineage Hematopoietic Neoplasm: A Case Report and Literature Review. Am J Leuk Res. 2023;6(1):1029..