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Myeloperoxidase deficiency and severe sepsis
N A Grossl1, A G Candel, A Shrit
1Department of Pathology, Loyola University Medical Center, Maywood, Ill.
Abstract:
We have described a 45-year-old obese white man found to have myeloperoxidase (MPO) deficiency of the granulocytic and monocytic series. Pancreatic necrosis due to bacterial infection developed as a complication of acute pancreatitis. Subsequently, he died of sepsis. MPO staining of terminal antemortem blood smears and postmortem bone marrow aspirates showed absence of MPO in cells of the myelocytic and monocytic series. Family members' neutrophils and monocytes stained positive for MPO. MPO deficiency associated with severe sepsis is rarely reported. This case serves as a review of the association between hereditary and acquired MPO deficiency and severe infection.
Insights
Myeloperoxidase (MPO) deficiency, an inherited immune disorder, was identified in a patient who developed severe sepsis and pancreatic necrosis. This case highlights the rare association between MPO deficiency and life-threatening infections.
Area of Science:
- Immunology
- Hematology
- Genetics
Background:
- Myeloperoxidase (MPO) is an enzyme crucial for neutrophil function in fighting bacterial infections.
- MPO deficiency is a genetic disorder characterized by the absence or reduced activity of this enzyme.
- While often asymptomatic, MPO deficiency can be associated with an increased risk of certain infections.
Observation:
- A 45-year-old obese male presented with acute pancreatitis complicated by bacterial infection, leading to pancreatic necrosis.
- The patient subsequently developed severe sepsis and ultimately died.
- Blood and bone marrow analyses confirmed the absence of MPO in granulocytic and monocytic cells, consistent with hereditary MPO deficiency.
Findings:
- The patient exhibited a complete absence of MPO in granulocytes and monocytes, indicating a hereditary MPO deficiency.
- Family members' cells stained positive for MPO, confirming the inherited nature of the deficiency.
- The case underscores the rare but serious link between MPO deficiency and susceptibility to severe bacterial infections like sepsis.
Implications:
- This case highlights the critical role of MPO in combating severe bacterial infections and preventing complications like sepsis.
- Understanding the interplay between MPO deficiency and infection risk is vital for clinical management and genetic counseling.
- Further research into the mechanisms underlying MPO deficiency-associated infections may reveal new therapeutic targets.