OVERVIEW

WHIM Syndrome (A Chronic Neutropenic Disorder): Uncouple the Complex for HCPs and Patients is organized by Global Oncology Academy (GOA).

Publication Dates
Release Date: 05/29/2024
Expiration Date: 05/29/2025

Description:
WHIM syndrome is a rare, inherited, combined immunodeficiency disease caused by reduced mobilization and trafficking of white blood cells from the bone marrow due to over-signaling of the CXCR4/CXCL12 pathway. WHIM syndrome is named for its four common clinical findings. The diagnostic challenges arise because not all symptoms are required for a diagnosis and all of them do not appear at the same time. Patients have deficient blood levels of neutrophils (neutropenia) and lymphocytes (lymphopenia), which results in frequent, recurrent infections. Affected individuals are particularly susceptible to human papillomavirus (HPV), which can cause skin and genital warts and potentially lead to certain types of cancer. Our program goal is to identify risk factors associated with WHIM syndrome, focusing on making an earlier diagnosis that can impact long-term outcomes, timely treatment options and ultimately improved quality of life for patients.

Learning Objectives:
After participating in this educational activity, participants should be better able to:
• Gain an understanding of WHIM syndrome as a rare PID/ chronic neutropenic disorder with diverse clinical presentations
• Implement strategic measures to improve the early identification of WHIM syndrome patients for prompt assessment and diagnosis to avoid potential complications and long-term sequelae
• Understand the CXCR4 pathway dysregulation and how it relates to the underlying causes of WHIM syndrome
• Garner an understanding of the limitations of current approaches for WHIM syndrome and potential new approaches for patients