Dapson Kullanımı İle Prezente Olan G6pd Enzim Eksikliğine Bağlı Hemolitik Anemi Vakası
Özet
Hemolitik anemi kandaki dolaşan eritrositlerin artan yıkımına bağlı sayıca azalmasıyla karakterizedir. Glukoz-6-Fosfat Dehidrogenaz (G6PD) eksikliği hemolizle ilişkili en sık görülen eritrosit enzim bozukluğudur. G6PD glutatyon metabolizmasında yer alarak eritrositleri oksidatif strese karşı koruyarak onların hemolize uğramasını önler. G6PD enzin eksikliğine bağlı hemolitik anemi yenidoğan sarılığı olarak görüldüğü gibi hayatın ilerleyen dönemlerinde enfeksiyonlara, favizme ve ilaçlara maruz kalındığında da oluşur. Dapson, pemfigus vulgaris gibi birçok cilt hastalığında hekimler tarafından tercih edilen ve G6PD eksikliğinde hemolize yol açan bir ilaçtır. İmmün olmayan bu hemoliz tipinde haptoglobulin düşük ve coombs testleri negatiftir. Daha sonra ortaya çıkabilecek ciddi bir komplikasyon olan hemolitik anemi, dapson tedavisine başlamadan önce G6PD enzim düzeylerine bakılarak önlenebilir. Bu olguda dapson kullanan bir hastada oluşan hemolitik aneminin G6PD eksikliğine bağlı olabileceğini vurgulamak istedik.
Referanslar
Wintrobe’s Clinical Hematology. Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, Eds. 10th edition. Mass Publishing Co., 1999.
Caleb J Scheckel, Ronald S Go. Autoimmune Hemolytic Anemia: Diagnosis and Differential Diagnosis. Hematol Oncol Clin North Am. 2022;36(2):315-324.
D Renard, A Rosselet.Drug-induced hemolytic anemia: Pharmacological aspects. Transfus Clin Biol. 2017 Sep;24(3):110-114.
Reclos GJ, Hatzidakis CJ, Schulpis KH. 2000. Glucose-6-phosphate dehyrogenase deficiency neonatal screening: preliminary evidence that a high percentage of partially deficient female neonates are missed during routine screening. Journal of Medical Screening. (7):1: 46-51.
Luzzatto L, Ally M, Notaro R. Glucose-6-phosphate dehydrogenase deficiency. Blood 2020;136:1225.
Beutler E. 2008. Glucose-6-phosphate dehydrogenase deficiency: a historical perspective. Blood, 11: 16-24.
Glader BE. 2008. Glucose-6-phosphate dehydrogenase deficiency and related disorders of hexose monophosphate shunt and glutathione metabolism. In: Wintrobe’s clinical hematology. 10th ed. Baltimore: Williams & Wilkins. p. 1176-90.
Glucose-6-phosphate dehydrogenase deficiency and hemolytic anemia. In: Nathan DG ,Orkin SH , eds. Nathan and Oski’s hematology of infancy and childhood. 7th ed. Philadelphia: W. B. Saunders, 2009: 883-900.
George Garratty, Patricia A Arndt. Drugs that have been shown to cause drug-induced immune hemolytic anemia or positive direct antiglobulin tests: some interesting findings since 2007. Immunohematology. 2014;30(2):66-79.
Glucose-6-phosphate dehydrogenase deficiency: a historical perspective. Blood 2008;11:16-24.
Krista Crider, Jennifer Williams, Yan Ping Qi et al. Folic acid supplementation and malaria susceptibility and severity among people taking antifolate antimalarial drugs in endemic areas.Cochrane Database Syst Rev. 2022; 2022(2): 1-19.
Ashurst JV, Wasson MN, Hauger W, Fritz WT. Pathophysiologic mechanisms, diagnosis, and management of dapsone-induced methemoglobinemia. J Am Osteopath Assoc. 2010;110:16-20.
Diagnosis and Management of G6PD Deficiency. Am Fam Physic, 2005, 72.
B R P Jonkers, G Cobanoglu, E J Blok, J J Köbben, M W van der Helm, J A M van Hoof, A N Tintu, C Bethlehem, J Versmissen. Symptomatic rebound methaemoglobinaemia after treatment with dapsone. Neth J Med. 2020 Sep;78(5):277-281.
Büyükokuroğlu ME, Süleyman H. Glukoz 6-fosfat dehidrogenaz eksikliği. Turkiye Klinikleri Journal of Medical Sciences. 2001;21:415-9
Garraty G. Immune hemolytic anemia associated with drug therapy. Blood Rev 2010;24:143-150
Referanslar
Wintrobe’s Clinical Hematology. Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, Eds. 10th edition. Mass Publishing Co., 1999.
Caleb J Scheckel, Ronald S Go. Autoimmune Hemolytic Anemia: Diagnosis and Differential Diagnosis. Hematol Oncol Clin North Am. 2022;36(2):315-324.
D Renard, A Rosselet.Drug-induced hemolytic anemia: Pharmacological aspects. Transfus Clin Biol. 2017 Sep;24(3):110-114.
Reclos GJ, Hatzidakis CJ, Schulpis KH. 2000. Glucose-6-phosphate dehyrogenase deficiency neonatal screening: preliminary evidence that a high percentage of partially deficient female neonates are missed during routine screening. Journal of Medical Screening. (7):1: 46-51.
Luzzatto L, Ally M, Notaro R. Glucose-6-phosphate dehydrogenase deficiency. Blood 2020;136:1225.
Beutler E. 2008. Glucose-6-phosphate dehydrogenase deficiency: a historical perspective. Blood, 11: 16-24.
Glader BE. 2008. Glucose-6-phosphate dehydrogenase deficiency and related disorders of hexose monophosphate shunt and glutathione metabolism. In: Wintrobe’s clinical hematology. 10th ed. Baltimore: Williams & Wilkins. p. 1176-90.
Glucose-6-phosphate dehydrogenase deficiency and hemolytic anemia. In: Nathan DG ,Orkin SH , eds. Nathan and Oski’s hematology of infancy and childhood. 7th ed. Philadelphia: W. B. Saunders, 2009: 883-900.
George Garratty, Patricia A Arndt. Drugs that have been shown to cause drug-induced immune hemolytic anemia or positive direct antiglobulin tests: some interesting findings since 2007. Immunohematology. 2014;30(2):66-79.
Glucose-6-phosphate dehydrogenase deficiency: a historical perspective. Blood 2008;11:16-24.
Krista Crider, Jennifer Williams, Yan Ping Qi et al. Folic acid supplementation and malaria susceptibility and severity among people taking antifolate antimalarial drugs in endemic areas.Cochrane Database Syst Rev. 2022; 2022(2): 1-19.
Ashurst JV, Wasson MN, Hauger W, Fritz WT. Pathophysiologic mechanisms, diagnosis, and management of dapsone-induced methemoglobinemia. J Am Osteopath Assoc. 2010;110:16-20.
Diagnosis and Management of G6PD Deficiency. Am Fam Physic, 2005, 72.
B R P Jonkers, G Cobanoglu, E J Blok, J J Köbben, M W van der Helm, J A M van Hoof, A N Tintu, C Bethlehem, J Versmissen. Symptomatic rebound methaemoglobinaemia after treatment with dapsone. Neth J Med. 2020 Sep;78(5):277-281.
Büyükokuroğlu ME, Süleyman H. Glukoz 6-fosfat dehidrogenaz eksikliği. Turkiye Klinikleri Journal of Medical Sciences. 2001;21:415-9
Garraty G. Immune hemolytic anemia associated with drug therapy. Blood Rev 2010;24:143-150
