findings in advSM

Q: In advanced systemic mastocytosis (advSM), impaired organ function will be described as? - select one

A) A findings

B) B findings 

C) C findings

Answer: C

There are five subtypes of SM:

• Indolent SM
• Smoldering SM
• Aggressive SM (ASM)
• Mast cell leukemia (MCL)
• SM with an associated hematologic/myeloid neoplasm (SM-AHN)

Last 3, i.e., ASM, MCL, and SM-AHN, are collectively referred to as advanced SM (advSM). 

B Findings - are defined as high disease burden or significant organ enlargement without organ dysfunction.

C Findings -  are defined as Impaired organ function due to mast cell infiltration.

#oncology-hematology

References:

1. Arber DA, Orazi A, Hasserjian RP, et al. International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood 2022; 140:1200.

2. Khoury JD, Solary E, Abla O, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia 2022; 36:1703.

3. Pardanani A, Reichard K, Tefferi A. Advanced systemic mastocytosis-Revised classification, new drugs and how we treat. Br J Haematol. 2024 Feb;204(2):402-414. doi: 10.1111/bjh.19245. Epub 2023 Dec 6. PMID: 38054381.

4. Ustun C, Keklik Karadag F, Linden MA, Valent P, Akin C. Systemic mastocytosis: current status and challenges in 2024. Blood Adv. 2025 May 13;9(9):2048-2062. doi: 10.1182/bloodadvances.2024012612. PMID: 39853317; PMCID: PMC12052678.