The Pathology of Systemic Mastocytosis: Classic Cases with Whole Slide Images Explained by Experts in the Field

Systemic mastocytosis (SM) is a rare hematologic neoplasm. Recent updates in SM classification and diagnostic criteria (WHO-5th edition and ICC) are being implemented in pathologists' practices. The rarity of the disease and variability of the clinical presentations pose many challenges for the diagnosis and correct subclassification of SM. Advances in laboratory testing for SM require pathologists to stay abreast with new techniques and have a clear understanding of technical advantages and pitfalls. Unfortunately, these challenges may significantly delay the diagnosis and negatively impact patient care as a correct diagnosis allows patients to be eligible for treatment by selective kinase inhibitors. Although many resources are available, including AIM Centers of Excellence/ Reference Centers which provide diagnostic and therapeutic services to such patients and the European Competence Network in Mastocytosis (ECNM), the initial recognition of SM often occurs at the level of a community pathologist.

This program consists of a series of 10 cases of SM and mimickers that had been reviewed by experts. Select whole slides, including special stains and immunohistochemical stains, have been scanned allowing for whole slide imaging which mimics the typical microscopic experience of a pathologist. This is embedded in an easy-to-use web-based program featuring annotated cases with relevant clinical and laboratory findings, followed by questions designed to test key concepts related to the diagnosis and classification of SM.

This activity was generously supported by an educational grant from Blueprint Medicines. Supporters of this educational activity have no influence over the content or recommendations contained within the activity.

Continuing Education

Learning Objectives

  1. Recognize key morphologic and immunophenotypic features of SM.
  2. Become familiar with key diagnostic laboratory tests (allele- specific KITD816V testing including high-sensitivity and NGS, high sensitivity flow cytometry) and understand their utility, advantages, and pitfalls.
  3. Be able to integrate the morphologic, laboratory, and clinical data to generate a diagnosis according to the recent WHO-5th edition and International Consensus Criteria (ICC) 2022 classifications and NCCN guidelines.

Accreditor language here. 10 CME credits available.