MPO deficiency confers impaired processing of neutrophil reactive oxygen species in a patient with severe CRMO

S Berg,H Björnsdottir,J Bylund,K Christenson,A Karlsson,M Sundqvist,V Osla,P Wekell,A Welin

doi:10.1186/1546-0096-13-s1-p191

Abstract

We report a severe case of chronic recurrent multifocal osteomyelitis (CRMO) associated with total myeloperoxidase (MPO) deficiency. The etiology of CRMO is in most cases unknown, and this is to our knowledge the first case associated with MPO-deficiency. Leukocyte MPO-deficiency renders neutrophils unable to process superoxide to secondary reactive oxygen species (ROS). Partial MPO deficiency is seldom associated with pathology but little is known about the effects of total MPO deficiency.

Highlights

We report a severe case of chronic recurrent multifocal osteomyelitis (CRMO) associated with total myeloperoxidase (MPO) deficiency
The etiology of CRMO is in most cases unknown, and this is to our knowledge the first case associated with MPO-deficiency
Partial MPO deficiency is seldom associated with pathology but little is known about the effects of total MPO deficiency

Summary

Open Access

S Berg1*, H Björnsdottir[2], M Sundqvist[2], P Wekell[1,3], K Christenson[2], V Osla[2], A Welin[2], J Bylund[4], A Karlsson2*. From 8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases Dresden, Germany. From 8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases Dresden, Germany. 30 September - 3 October 2015

Introduction

Patient and methods

Results

Conclusion