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Chronic Granulomatous Disease (CGD) and Complete Myeloperoxidase Deficiency Both Yield Strongly Reduced Dihydrorhodamine 123 Test Signals but Can Be Easily Discerned in Routine Testing for CGD

¹ University Clinic Carl Gustav Carus, Department of Pediatrics, Dresden, Germany.
² Institut für Laboratoriumsmedizin und Pathobiochemie, Berlin, Germany.
³ The Children’s Memorial Hospital, Chicago, IL.
⁴ Valley Medical Center, Lewiston, ID.
⁵ Department of Pediatrics, Charite, Berlin, Germany.
⁶ Max Planck Institute for Infection Biology, Berlin, Germany.
⁷ Department of Haematology, Technische Universität Dresden, Dresden, Germany.
⁸ Laboratoires Reunis Kutter-Lieners-Hastert, Luxembourg, Luxembourg.

^aAddress correspondence to this author at: University Clinic Carl Gustav Carus, Department of Pediatrics, Fetscherstrasse 74, 01307 Dresden, Germany. Fax: 49-351-458-6333; e-mail: roeslerj{at}rcs.urz.tu-dresden.de.

Background: The flow cytometric dihydrorhodamine 123 (DHR) assay is used as a screening test for chronic granulomatous disease (CGD), but complete myeloperoxidase (MPO) deficiency can also lead to a strongly decreased DHR signal. Our aim was to devise simple laboratory methods to differentiate MPO deficiency (false positive for CGD) and NADPH oxidase abnormalities (true CGD).

Methods: We measured NADPH-oxidase and MPO activity in neutrophils from MPO-deficient patients, CGD patients, NADPH-oxidase–transfected K562 cells and cells with inhibited and substituted MPO.

Results: Eosinophils from MPO-deficient individuals retain eosinophilic peroxidase and therefore generate a normal DHR signal. The addition of recombinant human MPO enhances the DHR signal when simply added to a suspension of MPO-deficient cells but not when added to NADPH-oxidase–deficient (CGD) cells. Lucigenin-enhanced chemiluminescence (LCL) is increased in neutrophils from MPO-deficient patients, whereas neutrophils from patients with CGD show a decreased response.

Conclusions: A false-positive result caused by MPO deficiency can be easily ascertained because, unlike cells from a CGD patient, cells from MPO-deficient patients (a) contain functionally normal eosinophils, (b) show a significant enhancement of the DHR signal following addition of rhMPO, and (c) generate a strong LCL signal.

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				G P Spickett Immune deficiency disorders involving neutrophils J. Clin. Pathol., September 1, 2008; 61(9): 1001 - 1005. [Abstract] [Full Text] [PDF]