Abstract
Aim: Myeloperoxidase (MPO) deficiency is the most common inherited defect of phagocytes. In this article, we aimed to reveal clinical characteristics of our patients with primary MPO deficiency.
Material and Method: In our study, patients aged 0-18 years, who were consulted to Ankara City Hospital Pediatric Hematology Department between 1 October 2019 and 1 December 2021 due to neutropenia, were retrospectively examined. If a patient had neutropenia in the complete blood count and inconsistently normal neutrophil count in the peripheral blood smear formula it was accepted as pseudoneutropenia. Patients with pseuduneutropenia were included in the study.
Results: Fifteen patients diagnosed with MPO deficiency were analyzed in the study. Nine of the patients were female, 6 were male, median age of the patients was 7 (0 – 17.5) years. The mean white blood cell (WBC) count of the patients was reported as 8219±2879/mm3, and the mean neutrophil count and percentage in the complete blood count printout was 33.30±15.88/mm3 and 0,74%±0, 94% respectively. The mean neutrophil count and percentage counted in the peripheral blood smear were 5186±1710 and 63.8%±10.59%, respectively. The mean LUC value on the complete blood count printout was 54.35%±19.47% (Normal range, 0-4%). In the flow cytometry evaluation of peripheral blood samples of the patients, it was observed that neutrophils were stained with CD33, CD13, CD16, CD11b monoclonal antibodies but not with MPO.
Conclusion: Peripheral smear evaluation is important when investigating the etiology of neutropenia. Many hematology analyzers using the MPO staining technique are indicative of MPO deficiency by identifying large unstained cells that do not stain with MPO. In patients who present with recurrent infections and MPO deficiency, other reasons that may predispose to infections should be investigated.
Supporting Institution
None
References
- Xiao X, Saha P, Yeoh BS, et al. Myeloperoxidase deficiency attenuates systemic and dietary iron-induced adverse effects. J Nutr Biochem 2018; 62: 28-34.
- Domingues-Ferreira M, Levy A, Barros NC, et al. Case report of myeloperoxidase deficiency associated with disseminated paracoccidioidomycosis and peritoneal tuberculosis. Rev Soc Bras Med Trop 2017; 50: 568-70.
- Albrett AM, Ashby LV, Dickerhof N, et al. Heterogeneity of hypochlorous acid production in individual neutrophil phagosomes revealed by a rhodamine-based probe. J Biol Chem 2018; 293: 15715-24.
- Yonezawa K, Horie O, Yoshioka A, et al. Association between the neutrophil myeloperoxidase index and subsets of bacterial infections. Int J Lab Hematol 2010; 32: 598-605.
- Marchetti C, Patriarca P, Solero GP, et al. Genetic characterization of myeloperoxidase deficiency in Italy. Hum Mutat 2004; 23: 496-505.
- Pahwa R, Modi P, Jialal I. Myeloperoxidase deficiency. 2021 Sep 28. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 29262241. Bookshelf ID: NBK470278,
- Nauseef WM. Diagnostic assays for myeloperoxidase and myeloperoxidase deficiency. Methods Mol Biol 2014; 1124: 537-46.
- Lanza F. Clinical manifestation of myeloperoxidase deficiency. J Mol Med 1998; 76: 676-81.
- Patıroğlu T, Güngör HE, Belohradsky JS, et al. Myeloperoxidase deficiency: the secret under the flag of unstained cell. Turkish J Hematol 2013; 3: 232
- Larrocha C, De Castro MF, Fontan G, et al. Hereditary myeloperoxidase deficiency: study of 12 cases. Scand J Haematol 1982; 29: 389–97.
- Cech P, Stalder H, Widmann JJ, et al. Leukocyte myeloperoxidase deficiency and diabetes mellitus associated with Candida albicans liver abscess. Am J Med 1979; 66: 149-53.
- Roh S, Ham JY, Song KE, et al. Myeloperoxidase deficiency manifesting as pseudoneutropenia with low mean peroxidase index and high monocyte count in 4 adult patients. Lab Med 2020; 51: 16-9.
- Gleghorn DM, Thomas W. Prevalence of myeloperoxidase deficiency determined using an ADVIA 2120i. Int J Lab Hematol 2020; 42: 220-3.
- Cakir I, Cakir N, Atalay MA, et al. Large unstained cells are correlated with inflammatory biomarkers in patients with invasive aspergillosis. Turkish J Biochem 2018; 43: 306–11.
- Kutter D, Devaquet P, Vanderstocken G, et al. Consequences of total and subtotal myeloperoxidase deficiency: risk or benefit? Acta Haematol 2000; 104: 10–5.
- Lanza F, Fietta A, Spisani S, et al. Does a relationship exist between neutrophil myeloperoxidase deficiency and the occurrence of neoplasms? J Clin Lab Immunol 1987; 22: 175-80.
- Telles RW, Ferreira GA, da Silva NP, et al. Increased plasma myeloperoxidase levels in systemic lupus erythematosus. Rheumatol Int 2010; 30: 779-84.
- Odobasic D, Kitching AR, Yang Y, et al. Neutrophil myeloperoxidase regulates T-cell-driven tissue inflammation in mice by inhibiting dendritic cell function. Blood 2013; 121: 4195–204.
- Aratani Y. Myeloperoxidase: Its role for host defense, inflammation, and neutrophil function. Arch Biochem Biophys 2018; 640: 47-52
Abstract
References
- Xiao X, Saha P, Yeoh BS, et al. Myeloperoxidase deficiency attenuates systemic and dietary iron-induced adverse effects. J Nutr Biochem 2018; 62: 28-34.
- Domingues-Ferreira M, Levy A, Barros NC, et al. Case report of myeloperoxidase deficiency associated with disseminated paracoccidioidomycosis and peritoneal tuberculosis. Rev Soc Bras Med Trop 2017; 50: 568-70.
- Albrett AM, Ashby LV, Dickerhof N, et al. Heterogeneity of hypochlorous acid production in individual neutrophil phagosomes revealed by a rhodamine-based probe. J Biol Chem 2018; 293: 15715-24.
- Yonezawa K, Horie O, Yoshioka A, et al. Association between the neutrophil myeloperoxidase index and subsets of bacterial infections. Int J Lab Hematol 2010; 32: 598-605.
- Marchetti C, Patriarca P, Solero GP, et al. Genetic characterization of myeloperoxidase deficiency in Italy. Hum Mutat 2004; 23: 496-505.
- Pahwa R, Modi P, Jialal I. Myeloperoxidase deficiency. 2021 Sep 28. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 29262241. Bookshelf ID: NBK470278,
- Nauseef WM. Diagnostic assays for myeloperoxidase and myeloperoxidase deficiency. Methods Mol Biol 2014; 1124: 537-46.
- Lanza F. Clinical manifestation of myeloperoxidase deficiency. J Mol Med 1998; 76: 676-81.
- Patıroğlu T, Güngör HE, Belohradsky JS, et al. Myeloperoxidase deficiency: the secret under the flag of unstained cell. Turkish J Hematol 2013; 3: 232
- Larrocha C, De Castro MF, Fontan G, et al. Hereditary myeloperoxidase deficiency: study of 12 cases. Scand J Haematol 1982; 29: 389–97.
- Cech P, Stalder H, Widmann JJ, et al. Leukocyte myeloperoxidase deficiency and diabetes mellitus associated with Candida albicans liver abscess. Am J Med 1979; 66: 149-53.
- Roh S, Ham JY, Song KE, et al. Myeloperoxidase deficiency manifesting as pseudoneutropenia with low mean peroxidase index and high monocyte count in 4 adult patients. Lab Med 2020; 51: 16-9.
- Gleghorn DM, Thomas W. Prevalence of myeloperoxidase deficiency determined using an ADVIA 2120i. Int J Lab Hematol 2020; 42: 220-3.
- Cakir I, Cakir N, Atalay MA, et al. Large unstained cells are correlated with inflammatory biomarkers in patients with invasive aspergillosis. Turkish J Biochem 2018; 43: 306–11.
- Kutter D, Devaquet P, Vanderstocken G, et al. Consequences of total and subtotal myeloperoxidase deficiency: risk or benefit? Acta Haematol 2000; 104: 10–5.
- Lanza F, Fietta A, Spisani S, et al. Does a relationship exist between neutrophil myeloperoxidase deficiency and the occurrence of neoplasms? J Clin Lab Immunol 1987; 22: 175-80.
- Telles RW, Ferreira GA, da Silva NP, et al. Increased plasma myeloperoxidase levels in systemic lupus erythematosus. Rheumatol Int 2010; 30: 779-84.
- Odobasic D, Kitching AR, Yang Y, et al. Neutrophil myeloperoxidase regulates T-cell-driven tissue inflammation in mice by inhibiting dendritic cell function. Blood 2013; 121: 4195–204.
- Aratani Y. Myeloperoxidase: Its role for host defense, inflammation, and neutrophil function. Arch Biochem Biophys 2018; 640: 47-52
Details
| Primary Language | English |
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| Subjects | Health Care Administration |
| Journal Section | Original Article |
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| Publication Date | March 15, 2022 |
| Published in Issue | Year 2022 Volume: 5 Issue: 2 |
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