We’re excited to share the International Consensus Classification System (ICC) and the World Health Organization (WHO) have acknowledged VEXAS to their classification systems for hematological cancers.
This is a very important step towards advancing global awareness and expediting the diagnosis of VEXAS and related autoimmune diseases, like relapsing polychondritis (RP).
The WHO and ICC Classification Systems are widely used by clinicians to render diagnoses, standardize the categorization of malignancies and guide treatments. They also assist public health officials and researchers with identifying the cause of specific cancers and monitoring their prevalence in the population.
We’d like to thank Dr. Rashmi Kanagal-Shamanna and her colleagues for advancing clinical knowledge regarding VEXAS, which ultimately resulted in its addition to two renowned classification systems. They wrote about it in a recent article entitled, “The 5th edition of the World Health Organization Classification of Haematolymphoid Tumors: Myeloid and Histiocytic/Dendritic Neoplasms” that was published in Leukemia.
The review summarizes the significant changes in the latest edition of the WHO classification system, which expands upon the previous (2017) guidelines by focusing on actionable biomarkers and incorporating new and/or rare disease entities into the classification schema.
Especially noteworthy to the autoimmune and hematopathology communities, is the acknowledgement and recognition of the close relationship between systemic inflammation, clonal somatic mutations and myeloid malignancies specifically based on data from emerging entities such as VEXAS with UBA1 mutations. The updated WHO classification, for the first time, formally incorporates precursor myeloid lesions such as clonal hematopoiesis (CH) into the classification system – further understanding of VEXAS and related precursor lesions is acknowledged.
What’s more, the concurrently released International Consensus Classification of Myeloid Neoplasms and Acute Leukemia goes a step further by listing VEXAS under the spectrum of pre-malignant “clonal cytopenias” alongside clonal cytopenia of undetermined significance (CCUS) and myelodysplastic syndrome (MDS).
“The inclusion of VEXAS in the classification systems used for hematological cancers is extremely important,’” said Dr. Kanagal-Shamanna, Hematopathologist and Molecular Genetic Pathologist at MD Anderson Cancer Center, “It increases awareness of VEXAS among clinicians and pathologists and facilitates prompt diagnosis thereby promoting a holistic approach to patient care, from diagnosis to post-diagnosis management.”
“The RP Foundation is pleased to invest in RP- and related autoimmune research projects, like VEXAS, that are identifying the genetic basis of autoimmune diseases and move science closer to the discovery of actionable biomarkers,” said David Bammert, President of the RP Foundation, “It’s exciting to know that the research that we’re investing in will potentially make a significant impact in the diagnosis and care of patients across the spectrum of diseases.”
Where there’s research, there’s hope. Learn more about our research initiatives or donate today. Together, we can fuel autoimmune disease research and bring hope to patients with RP.