Aksiller Lenfadenopati: Tek Bir Lenfadenopati ile Kronik Granülomatöz Hastalık Tanısı Alan Çocuk Hasta

Year 2019, Volume: 9 Issue: 3, 217 - 220, 01.12.2019

Pınar Gür Çetinkaya Deniz Çağdaş Ayvaz İlhan Tezcan

Abstract

Lenfadenit, kronik granülomatöz hastalığın (KGH) en belirgin bulgularından biridir. Katalaz pozi-tif mikroorganizmalarla tekrarlayan enfeksiyonlar, bu hastalarda mortalite ve morbiditeye neden olurlar. Hematopoetik kök hücre naklinden (HKHN) sonra pek çok komplikasyon görülmesine rağmen, KGH’deki tek kesin tedavi yöntemidir. Bu çalışmadaki olgumuz, persistan sol aksiller lenfadenopati ile başvurmuş olup bu sayede X’e bağlı KGH tanısı almıştır. Hastaya HLA tam uyumlu aile dışı vericiden başka bir merkezde HKHN yapıldı ve tam donör kimerizm sağlandı.

Keywords

kronik granülomatöz hastalık; lenfadenopati; kök hücre nakli

References

• 1. Segal BH, Leto TL, Gallin JI, Malech HL, Holland SM. Genetic, biochemical, and clinical features of chronic granulomatous disease. Medicine 2000;79(3):170–200.
• 2. Winkelstein JA, Marino MC, Johnston Jr RB, Boyle J, Curnutte J, Gallin JI, et al. Chronic granulomatous disease: report on a national registry of 368 patients. Medicine 2000;79(3):155–69.
• 3. Matute JD, Arias AA, Wright NA, Wrobel I, Waterhouse CC, Li XJ, et al. A new genetic subgroup of chronic granulomatous disease with autosomal recessive mutations in p40phox and selective defects in neutrophil NADPH oxidase activity. Blood 2009;114(15):3309–15.
• 4. Greenberg DE, Ding L, Zelazny AM, Stock F, Wong A, Anderson VL, et al. A novel bacterium associated with lymphadenitis in a patient with chronic granulomatous disease. PLoS Pathog 2006;2(4):260–7.
• 5. Kulkarni M, Desai M, Gupta M, Dalvi A, Taur P, Terrance A, et al. Clinical, Immunological, and Molecular Findings of Patients with p47phox Defect Chronic Granulomatous Disease (CGD) in Indian Families. J Clin Immunol 2016;36(8):774–84.
• 6. Van den Berg JM, Van Koppen E, Åhlin A, Belohradsky BH, Bernatowska E, Corbeel L, et al. Chronic granulomatous disease: the European experience. PloS one 2009;4(4):1–10.
• 7. Seger RA. Hematopoietic stem cell transplantation for chronic granulomatous disease. Immunol Allergy Clin North Am 2010;30(2):195–208.
• 8. Åhlin A, Boer M, Roos D, Leusen J, Smith C, Sundin U, et al. Prevalence, genetics and clinical presentation of chronic granulomatous disease in Sweden. Acta Paediatr 1995;84(12):1386–94.
• 9. Kuhns DB, Alvord WG, Heller T, Feld JJ, Pike KM, Marciano BE, et al. Residual NADPH oxidase and survival in chronic granulomatous disease. N Engl J Med 2010;363(27):2600–10.
• 10. Bortoletto P, Lyman K, Camacho A, Fricchione M, Khanolkar A, Katz BZ. Chronic granulomatous disease: a large, singlecenter US experience. Pediatr Infect Dis J 2015;34(10):1110–4.
• 11. Seger RA, Gungor T, Belohradsky BH, Blanche S, Bordigoni P, Di Bartolomeo P, et al. Treatment of chronic granulomatous disease with myeloablative conditioning and an unmodified hemopoietic allograft: a survey of the European experience, 1985–2000. Blood 2002;100(13):4344–50.
• 12. Horwitz ME, Barrett AJ, Brown MR, Carter CS, Childs R, Gallin JI, et al. Treatment of chronic granulomatous disease with nonmyeloablative conditioning and a T-cell-depleted hematopoietic allograft. N Engl J Med 2001;344(12):881–8.
• 13. Schuetz C, Hoenig M, Gatz S, Speth F, Benninghoff U, Schulz A, et al. Hematopoietic stem cell transplantation from matched unrelated donors in chronic granulomatous disease. Immunol Res 2008;44(1–3):35–41.