PriMera Scientific Surgical Research and Practice (ISSN: 2836-0028)

Editorial

Volume 1 Issue 4

The Ideological Submersion-Systemic Mastocytosis

Anubha Bajaj*

February 28, 2023

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Abstract

     Systemic mastocytosis is a heterogeneous disorder characteristically demonstrating mast cells with aberrant cytology and immuno-phenotype accumulated within diverse organs. The condition exhibits an anomalous pattern of neoplastic evolution.

World Health Organization (WHO) classification 2008 denominates distinct categories of mastocytosis as

  • Cutaneous mastocytosis.
  • Indolent systemic mastocytosis with minimal burden of mast cells and absence of cutaneous lesions.
  • Systemic mastocytosis associated with clonal hematologic non mast cell lineage disease as myelodysplastic syndrome (MDS), myeloproliferative neoplasm(MPN), acute myeloid leukaemia(AML) or lymphoma.
  • Aggressive systemic mastocytosis exemplifying diagnostic criteria of systemic mastocytosis, absence of mast cell leukaemia or incrimination of cutaneous surfaces.
  • Mast cell leukaemia with mast cells configuring > 20% of nucleated marrow cells or an aleukemic variant with < 10% of circulating white blood cells as mast cells.
  • Extra-cutaneous mastocytoma constituted of unifocal mast cell tumour demonstrating a non destructive neoplastic configuration.
  • Mast cell sarcoma is articulated of unifocal neoplasm exhibiting a destructive tumour configuration and constituent mast cells delineating aggressive cytological features [1, 2].

References

  1. Ungerstedt J., et al. “Clinical Outcomes of Adults with Systemic Mastocytosis: A 15-Year Multidisciplinary Experience”. Cancers (Basel) 1416 (2022): 3942.
  2. Valent P., et al. “Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal”. Hemasphere 5.11 (2021): e646.
  3. Leguit RJ., et al. “The international consensus classification of mastocytosis and related entities”. Virchows Arch (2022).
  4. El Hussein S., et al. “Systemic Mastocytosis and Other Entities Involving Mast Cells: A Practical Review and Update”. Cancers (Basel) 14.14 (2022): 3474.
  5. Sciumè M., et al. “Target Therapies for Systemic Mastocytosis: An Update”. Pharmaceuticals (Basel) 15.6 (2022): 738.
  6. Image 1 Courtesy: ASH.com.
  7. Image 2 Courtesy: Science direct.