Immune Dysregulation Program News

CHOP hosts the 7th Annual Immune Dysregulation Symposium on April 30 for clinicians and researchers who care for patients with complex, multi-organ immune diseases.

Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are overlapping hyperinflammatory syndromes that involve disorganization and malfunction of the immune system, leading to widespread inflammation and organ damage. The conditions can progress rapidly, making early identification and management key for preventing organ failure and death.

A CHOP-led study has shown that a regulatory class of T cells descends from two different origins, one that relates to autoimmunity and one that relates to protective immunity.

We all learned some simple patterns for diseases of the immune system when we were in training: immunodeficiency is “too little immune system,” autoimmunity is “too much immune system,” atopy is a “allergic immune system,” and lymphoproliferation is “a rapidly expanding immune system.”

CHOP researchers found that levels of proteins in the plasma of patients with MIS-C are significantly dysregulated and share features with two other inflammatory syndromes.

Using a model developed at CHOP, researchers find high cost of treatment most likely to prevent premature death in patients with agammaglobulinemia undermines its benefits.

Since March 2020, CHOP researchers have played a vital role in combatting the COVID-19 pandemic. Read highlights of their game-changing work.

CHOP researchers discovered a new condition, which they named PU.MA, and treated a patient with a bone marrow donation from the patient’s older brother.

Using deep immune profiling, CHOP researchers have found children with MIS-C are extremely immune activated, more like adults with severe COVID-19.

CHOP researchers found the elevated biomarker in all children who tested positive for SARS-CoV-2, whether they were symptomatic for COVID-19 or not.