OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press13810.17650/1818-8346-2015-1-46-52BASIC RESEARCHФУНДАМЕНТАЛЬНЫЕ ИССЛЕДОВАНИЯ В ПРАКТИЧЕСКОЙ МЕДИЦИНЕ НА СОВРЕМЕННОМ ЭТАПЕNeutropenia in pediatric hematology/oncology practiceНейтропении в практике детского гематолога/онкологаDeordievaE. A.ДеордиеваЕ. А.
Russian Federation
deor2005@yandex.ruShcherbinaA. Yu.ЩербинаА. Ю.
Russian Federation
Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of RussiaФГБУ ФНКЦ ДГОИ им. Дмитрия Рогачева Минздрава России1 Samory Mashela St., Moscow, 117198, RussiaРоссия, 117198, Москва, ул. Саморы Машела, 10406201510146520406201504062015https://oncohematology.abvpress.ru/ongm/article/view/138

Acquired neutropenia is one of the most common conditions in pediatric hematology practice. These conditions usually are benign. In contrast, congenital neutropenia are rare conditions, but in the absence of pathogenic therapy can cause fatal complications. Approach to the differential diagnosis and management of these patients are discussed in this review.

Приобретенная нейтропения – одно из самых частых состояний в практике детского гематолога. Эти состояния, как правило, протекают благоприятно. В отличие от них врожденные нейтропении – состояния достаточно редкие, но в отсутствие патогенетической терапии чреваты смертельными осложнениями. Подходы к дифференциальной диагностике этих состояний, ведению таких больных обсуждаются в данном обзоре.

acquired neutropeniacongenital neutropeniagranulocyte colony-stimulating factorimmunizationнейтропения приобретеннаянейтропения врожденнаягранулоцитарный колониестимулирующий факторвакцинация1. Гематология/онкология детского возраста. Под ред. А. Г. Румянцева и Е. В. Самочатовой. М.: Медпрактика-М, 2004. [Pediatric hematology/oncology. Ed.: Rumyantsev A. G., Samochatova E. V. M.: Medpraktika-M, 2004. (In Russ.)].Гематология/онкология детского возраста. Под ред. А. Г. Румянцева и Е. В. Самочатовой. М.: Медпрактика-М, 2004. [Pediatric hematology/oncology. Ed.: Rumyantsev A. G., Samochatova E. V. M.: Medpraktika-M, 2004. (In Russ.)].2. Bohn G., Welte K., Klein C. Severe congenital neutropenia: new genes explain an old disease. Curr Opin Rheumatol 2007;19(6):644–50.Bohn G., Welte K., Klein C. Severe congenital neutropenia: new genes explain an old disease. Curr Opin Rheumatol 2007;19(6):644–50.3. Andersohn F., Konzen C., Garbe E. Systematic review: agranulocytosis induced by nonchemotherapy drugs. Ann Intern Med 2007;146(9):657–65.Andersohn F., Konzen C., Garbe E. Systematic review: agranulocytosis induced by nonchemotherapy drugs. Ann Intern Med 2007;146(9):657–65.4. Bux J., Behrens G., Jaeger G., Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood 1998;91(1):181–6.Bux J., Behrens G., Jaeger G., Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood 1998;91(1):181–6.5. Володин Н. Н., Румянцев А. Г., Щербина А. Ю. Диагностика и лечение нейтропении у новорожденных. Вопросы практической педиатрии 2012;7(1):33–9. [Volodin N. N., Rumyantsev A. G., Shcherbina A. Yu. Diagnosis and treatment of neutropenia in newborns. Voprosy prakticheskoy pediatrii = Issues of Practical Pediatrics 2012;7(1):33–9. (In Russ.)].Володин Н. Н., Румянцев А. Г., Щербина А. Ю. Диагностика и лечение нейтропении у новорожденных. Вопросы практической педиатрии 2012;7(1):33–9. [Volodin N. N., Rumyantsev A. G., Shcherbina A. Yu. Diagnosis and treatment of neutropenia in newborns. Voprosy prakticheskoy pediatrii = Issues of Practical Pediatrics 2012;7(1):33–9. (In Russ.)].6. Autrel-Moignet A., Lamy T. Autoimmune neutropenia. Presse Med 2014; 43(4 Pt 2):105–18.Autrel-Moignet A., Lamy T. Autoimmune neutropenia. Presse Med 2014; 43(4 Pt 2):105–18.7. Walkovich K., Boxer L. A. How to approach neutropenia in childhood. Pediatr Rev 2013;34(4):173–84.Walkovich K., Boxer L. A. How to approach neutropenia in childhood. Pediatr Rev 2013;34(4):173–84.8. Boztug K., Klein C. Genetic etiologies of severe congenital neutropenia. Curr Opin Pediatr 2011;23(1):21–6.Boztug K., Klein C. Genetic etiologies of severe congenital neutropenia. Curr Opin Pediatr 2011;23(1):21–6.9. Kostmann R. Infantile genetic agranulocytosis; agranulocytosis infantilis hereditaria. Acta aediatr Suppl 1956;45(Suppl 105):1–78.Kostmann R. Infantile genetic agranulocytosis; agranulocytosis infantilis hereditaria. Acta aediatr Suppl 1956;45(Suppl 105):1–78.10. Klein C., Grudzien M., Appaswamy G. et al. HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease). Nat Genet 2007;39(1):86–92.Klein C., Grudzien M., Appaswamy G. et al. HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease). Nat Genet 2007;39(1):86–92.11. Европейский регистр тяжелой хронической нейтропении. Электронный ресурс. Доступ: http://www.severe-chronicneutropenia.org/forms/Europ_Prot. pdf. [European Register of Severe Chronic Neutropenia. Online resource. Access: http://www.severe-chronic-neutropenia.org/forms/Europ_Prot. pdf].Европейский регистр тяжелой хронической нейтропении. Электронный ресурс. Доступ: http://www.severe-chronicneutropenia.org/forms/Europ_Prot. pdf. [European Register of Severe Chronic Neutropenia. Online resource. Access: http://www.severe-chronic-neutropenia.org/forms/Europ_Prot. pdf].12. Динова Е. А., Курникова М. А., Масчан М. А. и др. Клинические и молекулярно-генетические особенностигруппы больных с врожденной нейтропенией. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2012;4:12–9. [Dinova E. A., Kurnikova M. A., Maschan M. A. et al. Clinical and genetic features of patients with congenital neutropenia. Voprosy gematologii/onkologii i immunopatologii v pediatrii = ediatric Hematology/ Oncology and Immunopathology 2012;4:12–9. (In Russ.)].Динова Е. А., Курникова М. А., Масчан М. А. и др. Клинические и молекулярно-генетические особенностигруппы больных с врожденной нейтропенией. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2012;4:12–9. [Dinova E. A., Kurnikova M. A., Maschan M. A. et al. Clinical and genetic features of patients with congenital neutropenia. Voprosy gematologii/onkologii i immunopatologii v pediatrii = ediatric Hematology/ Oncology and Immunopathology 2012;4:12–9. (In Russ.)].13. Dale D. C., Person R. E., Bolyard A. A. et al. Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia. Blood 2000;96(7):2317–22.Dale D. C., Person R. E., Bolyard A. A. et al. Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia. Blood 2000;96(7):2317–22.14. Динова Е. А., Щербина А. Ю., Румянцев А. Г. Тяжелая врожденная нейтропения. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2011;10(3):5–10. [Dinova E. A., Shcherbina A. Yu., Rumyantsev A. G. Severe congenital neutropenia. Voprosy gematologii/onkologii i immunopatologii v pediatrii = Pediatric Hematology/ Oncology and Immunopathology 2011;10(3):5–10. (In Russ.)].Динова Е. А., Щербина А. Ю., Румянцев А. Г. Тяжелая врожденная нейтропения. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2011;10(3):5–10. [Dinova E. A., Shcherbina A. Yu., Rumyantsev A. G. Severe congenital neutropenia. Voprosy gematologii/onkologii i immunopatologii v pediatrii = Pediatric Hematology/ Oncology and Immunopathology 2011;10(3):5–10. (In Russ.)].15. Welte К., Zeidler С. Severe congenital neutropenia. Hematol Oncol Clin North Am 2009;23(2):307–20.Welte К., Zeidler С. Severe congenital neutropenia. Hematol Oncol Clin North Am 2009;23(2):307–20.16. Dale D. C., Welte K. Cyclic and chronic neutropenia. Cancer Treat Res 2011; 157:97–108.Dale D. C., Welte K. Cyclic and chronic neutropenia. Cancer Treat Res 2011; 157:97–108.17. Bonilla M. A., Gillio A. P., Ruggeiro M. et al. Effects of recombinant human granulocyte colony-stimulating factor on neutropenia in patients with congenital agranulocytosis. N Engl J Med 1989;320(24):1574–80.Bonilla M. A., Gillio A. P., Ruggeiro M. et al. Effects of recombinant human granulocyte colony-stimulating factor on neutropenia in patients with congenital agranulocytosis. N Engl J Med 1989;320(24):1574–80.18. Dale D. C., Bonilla M. A., Davids M. W. et al. A randomized controlled phase III trial of recombinant human granulocyte colonystimulating factor (filgrastim) for treatment of severe congenital neutropenia. Blood 1993;81(10):2496–502.Dale D. C., Bonilla M. A., Davids M. W. et al. A randomized controlled phase III trial of recombinant human granulocyte colonystimulating factor (filgrastim) for treatment of severe congenital neutropenia. Blood 1993;81(10):2496–502.19. Grigull L., Pulver N., Goudeva L. et al. G-CSF mobilised granulocyte transfusions in 32 paediatric patients with neutropenic sepsis. Support Care Cancer 2006;14(9):910–6.Grigull L., Pulver N., Goudeva L. et al. G-CSF mobilised granulocyte transfusions in 32 paediatric patients with neutropenic sepsis. Support Care Cancer 2006;14(9):910–6.20. Ferry C., Ouachée M., Leblanc T. et al. Hematopoietic stem cell transplantation in severe congenital neutropenia: experience of the French SCN register. Bone Marrow Transplant 005; 35(1):45–50.Ferry C., Ouachée M., Leblanc T. et al. Hematopoietic stem cell transplantation in severe congenital neutropenia: experience of the French SCN register. Bone Marrow Transplant 005; 35(1):45–50.21. Dale D. C., Cottle T. E., Fier C. J. et al. Severe chronic neutropenia: treatment and follow-up of patients in the Severe Chronic Neutropenia International Registry. Am J Hematol 2003;72(2):82–93.Dale D. C., Cottle T. E., Fier C. J. et al. Severe chronic neutropenia: treatment and follow-up of patients in the Severe Chronic Neutropenia International Registry. Am J Hematol 2003;72(2):82–93.22. Freedman M. H., Alter B. P. Malignant myeloid transformation in congenital forms of neutropenia. Isr Med Assoc J 2002;4(11):1011–4.Freedman M. H., Alter B. P. Malignant myeloid transformation in congenital forms of neutropenia. Isr Med Assoc J 2002;4(11):1011–4.23. Rosenberg P. S., Alter B. P., Bolyard A. A. t al.; Severe Chronic Neutropenia International Registry. The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy. Blood 2006;107(12):4628–35.Rosenberg P. S., Alter B. P., Bolyard A. A. t al.; Severe Chronic Neutropenia International Registry. The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy. Blood 2006;107(12):4628–35.24. Makaryan V., Zeidler C., Bolyard A. A. et al. The diversity of mutations and clinical outcomes for ELANE-associated neutropenia. Curr Opin Hematol 2015;22(1):3–11.Makaryan V., Zeidler C., Bolyard A. A. et al. The diversity of mutations and clinical outcomes for ELANE-associated neutropenia. Curr Opin Hematol 2015;22(1):3–11.25. Европейский регистр тяжелой хронческой нейтропении – руководство по ведению больных. Электронный ресурс. Доступ: http://www.severe-chronicneutropenia. org/handbooks/handbook_en. pdf. [European Register of Severe Chronic Neutropenia. Guidelines for patient management. Online resource. Access: http://www.severe-chronic-neutropenia.org/handbooks/handbook_en. pdf].Европейский регистр тяжелой хронческой нейтропении – руководство по ведению больных. Электронный ресурс. Доступ: http://www.severe-chronicneutropenia. org/handbooks/handbook_en. pdf. [European Register of Severe Chronic Neutropenia. Guidelines for patient management. Online resource. Access: http://www.severe-chronic-neutropenia.org/handbooks/handbook_en. pdf].26. Weber D. J., Rutala W. A. Immunization of immunocompromised persons. Immunol Allergy Clin North Am 2003;23(4):605–34.Weber D. J., Rutala W. A. Immunization of immunocompromised persons. Immunol Allergy Clin North Am 2003;23(4):605–34.27. Centers for Disease Control and Prevention. General Recommendations on Immunization. Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep 2011;60(RR-02):1–61.Centers for Disease Control and Prevention. General Recommendations on Immunization. Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep 2011;60(RR-02):1–61.28. Canadian Immunization Guide. http://www.phac-aspc.gc.ca/publicat/cig- gci/index-eng. php.Canadian Immunization Guide. http://www.phac-aspc.gc.ca/publicat/cig- gci/index-eng. php.29. Australian Immunization Handbook.http://www.health.gov.au/internet/ immunise/publishing. nsf/Content/Handbook10-home.Australian Immunization Handbook.http://www.health.gov.au/internet/ immunise/publishing. nsf/Content/Handbook10-home.