Case Reports Hematopathology 2022 Exercise 2: VEXAS Syndrome: A Newly Described Systemic Inflammatory Syndrome with Hematologic Manifestations and Vacuoles

Authors

Qi Cao, MD, PhD

Department of Pathology

Los Angeles County University of Southern California Medical Center

Ashley Satsuki Hagiya, MD

Department of Pathology

Keck School of Medicine of the University of Southern California

Primary Audience: Pathologists and Clinical Scientists
Secondary Audience: Residents, Laboratory Technologist/Scientist (Includes all sub-specialty areas), Students, Pathologist Assistants,  Laboratory Directors and Educators

Upon completion of this activity, you will be able to:

·       describe the clinical features of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome;

·       recognize the characteristic bone marrow findings seen in VEXAS syndrome;

·       identify diagnostic considerations when cytoplasmic vacuoles are observed;

·       discuss the genetic basis of VEXAS syndrome; and

·       compile clinicopathologic data to make a diagnosis of VEXAS syndrome.