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Year 2020, Volume: 3 Issue: 1, 16 - 21, 30.04.2020

Halil Çelik , Canan Albayrak Davut Albayrak

Abstract

References

  • 1) Nathalie A, Guy L, Thierry L, Marie Q P, Gérard M, Yves B, et al. ,for the Centre de Référence National des Cytopénies Auto-immunes de l’Enfant (CEREVANCE).New insights into childhood autoimmune hemolytic anemia:a French national observational study of 265 children. Haematologica. 2011; 96(5): 655–663.
  • 2) Buchanan GR, Boxer LA, Nathan DG. The acute and transient nature of idiopathicimmune hemolytic anemia in childhood. J Pediatr. 1976;88(5):780-3.
  • 3) Habibi B, Homberg JC, Schaison G, Salmon C. Autoimmune hemolytic anemia in children.A review of 80 cases. Am J Med.1974;56(1):61-9.
  • 4) Heisel MA, Ortega JA. Factors influencing prognosis in childhood autoimmune hemolytic anemia. Am J Pediatr Hematol Oncol. 1983;5(2):147-52.
  • 5) Sokol RJ, Hewitt S, Stamps BK, Hitchen PA. Autoimmune haemolysis in childhood andadolescence. Acta Haematol. 1984;72(4):245-57.
  • 6) Zuelzer WW, Mastrangelo R, Stulberg CSPoulik MD, Page RH, Thompson RI.Autoimmune hemolytic anemia. Natural history and viral-immunologic interactions in childhood. Am J Med. 1970;49(1):80-93.
  • 7) Blackall DP. Warm-reactive autoantibodies in pediatric patients: clinical and serologic correlations. J Pediatr Hematol Oncol.2007;29(11):792-6.
  • 8) Naithani R, Agrawal N, Mahapatra M,Kumar R, Pati HP, Choudhry VP.Autoimmune hemolytic anemia in children.Pediatr Hematol Oncol. 2007;24(4):309-15.
  • 9) Oliveira MC, Oliveira BM, Murao M, Vieira ZM, Gresta LT, Viana MB. Clinicalcourse of autoimmune hemolytic anemia:an observational study. J Pediatr. (Rio J)2006;82(1):58-62.
  • 10) Vaglio S, Arista MC, Perrone MP, Tomei G,Testi AM, Coluzzi S, et al. Autoimmune hemolytic anemia in childhood: serologicfeatures in 100 cases. Transfusion. 2007;47(1):50-4.
  • 11) Evans RS, Takahashi K, Duane RT, Payne R,Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951;87(1):48-65
  • 12) Mathew P, Chen G, Wang W. Evans syndrome:results of a national survey. J Pediatr Hematol Oncol. 1997;19(5):433-7.
  • 13) Ng SC. Evans syndrome: a report on 12 patients. Clin Lab Haematol. 1992;14(3):189-93.
  • 14) Pui CH, Wilimas J, Wang W. Evans syndrome in chil18. Wang WC. Evans syndrome in childhood:pathophysiology, clinical course, and treatment.Am J Pediatr
  • 15) Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans' syndrome. Arch Dishild. 1997;77(3):245-8.
  • 16) Nelson DA, Davey FR. Erytrocytic disorders. In:Henry JB(Ed.). Clinical diagnosis and management by laboratory methods. 18th ed. Philadelphia: WB Saunders Co;1991;ch26,660-2
  • 17) Sarper N, Kılıc S, Zengin E, Gelen S A. Management of autoimmune hemolytic anemia in children and adolescents: A single center experience. Turk J Hematol 2011; 28: 198-205.
  • 18) Ware RE.Autoimmune hemolytic anemia In:Nathan DG,Orkin SH,Ginsburg D,Look AT eds.Nathan and Oski’s Hematology of Infancy and Chıldhood.6th ed.Philadelphia:W.B.Saunders;2003; 1:521-594.
  • 19) Pui, C., Wilimas, J. & Wang, W. Evans syndrome in childhood.The Journal of Pediatrics, 1980;97: 754–758.
  • 20) Wang, W. (1988) Evans syndrome in childhood: pathophysiology,clinical course, and treatment. The American Journal of PediatricHematology/Oncology, 10, 330–338.
  • 21) Mathew, P., Chen, G. & Wang, W. Evans Syndrome: results of anational survey. Journal of Pediatric Hematology/Oncology, 1997;19: 433–437.
  • 22) Savasan, S, Warrier, I. & Ravindranath, Y. The spectrumof Evans’ syndrome. Archives of Disease in Childhood, 1997;77: 245–248.
  • 23)Teachey, D.T., Manno, C.S., Axsom, K.M., Andrews, T., Choi, J.K.,Greenbaum, B.H., et all Unmasking Evans syndrome: T-cell phenotypeand apoptotic response reveal autoimmune lymphoproliferativesyndrome (ALPS). Blood, 2005;105: 2443–244

Otoimmun Hemolitik Anemi ve Evans Sendromlu Çocuk Hastalarda Klinik Özelliklerin Değerlendirilmesi

Year 2020, Volume: 3 Issue: 1, 16 - 21, 30.04.2020

Halil Çelik , Canan Albayrak Davut Albayrak

Abstract

Amaç: Otoimmun Hemolitik
Anemiler (OİHA) eritrosit yüzey membranına bağlanan otoantikorlar sonucu
eritrositlerin erken parçalanması ile karakterizedirler. Evans Sendromu (ES), OİHA
geliştiği sırada veya anemi geliştikten sonra immün trombositopeni gelişen bir
hemolitik anemidir. Bu çalışmada OİHA ve ES tanısı ile izlenen çocuk hastaların
demografik, klinik ve laboratuvar özelliklerinin, klinik seyirlerinin, tedaviye
cevaplarının, ve prognozlarına etki eden faktörlerin değerlendirilmesin
amaçlandık.



Yöntem: Haziran 2006- Haziran 2011 tarihleri
arasında Ondokuzmayıs Üniversitesi Çocuk Hematoloji Bilim Dalı’nda takip edilen
OİHA ve ES tanılı hastaların dosyaları geriye
dönük olarak incelendi. Otoimmun
hemolitik anemi tanısı, aynı zaman diliminde hemoglobin konsantrasyonu
11g/dl’den düşük,  direkt antiglobin
testi (DAT) pozitif ve periferik yaymada hemoliz bulgusu varlığının üçünün de
bulunduğu  hastalara konuldu. Talasemi
major hastaları, kemik iliği transplantasyonu (KİT) sonrası gelişen hemolitik
anemiler ve yenidoğan döneminde görülen Rh ve ABO uygunsuzluğuna bağlı
hemolitik anemiler çalışmaya dahil edilmedi. Bu hastaların demografik, klinik ve laboratuvar özellikleri, etiyolojide
rol oynayan faktörleri, klinik seyirleri, tedaviye cevapları ve  sağ kalımları kaydedildi.



Bulgular: Çalışmamıza
OİHA ve ES tanısı olan yaş ortancası 36 (12-44) [ortanca (ÇAA)] ay olan, 21
(%57’si erkek) hasta dahil edildi. Evans sendromlu 7 olgudan 4’ünde  (%57) ‘çift negatif T lenfosit’ pozitifliği
olduğu için bu hastalar otoimmün lenfoproliferatif sendrom (OLPS) tanısı alarak
çalışma dışı bırakıldı. Yirmi bir hastanın 18’i (%85) OİHA, 3’ü (%15) ES idi.
OİHA’lı hastaların 13’ü (%72) primer OİHA 5’i (%28) sekonder OİHA idi. İzole OİHA
18 hastamızın tedaviye cevap durumu incelendiğinde; 16’sının (%88) tam
remisyona girip tedavisinin kesildiği, 1’inin (%6) halen tedavi almakta olduğu,
1’inin ise (%6) eksitus olduğu tespit edildi.



Tartışma: Tam remisyona giren
hastalarımızın yaklaşık yarısı standart steroid tedavisi ile remisyona girememiş
ve bu hastalara  ilave  diğer tedaviler verilmiştir.  Otoimmun hemolitik anemiler ve ES çocukluk
çağında nadir görülmesine rağmen mortalite ve morbiditeleri fazladır. 

Keywords

çocukluk çağı evans sendromu

References

  • 1) Nathalie A, Guy L, Thierry L, Marie Q P, Gérard M, Yves B, et al. ,for the Centre de Référence National des Cytopénies Auto-immunes de l’Enfant (CEREVANCE).New insights into childhood autoimmune hemolytic anemia:a French national observational study of 265 children. Haematologica. 2011; 96(5): 655–663.
  • 2) Buchanan GR, Boxer LA, Nathan DG. The acute and transient nature of idiopathicimmune hemolytic anemia in childhood. J Pediatr. 1976;88(5):780-3.
  • 3) Habibi B, Homberg JC, Schaison G, Salmon C. Autoimmune hemolytic anemia in children.A review of 80 cases. Am J Med.1974;56(1):61-9.
  • 4) Heisel MA, Ortega JA. Factors influencing prognosis in childhood autoimmune hemolytic anemia. Am J Pediatr Hematol Oncol. 1983;5(2):147-52.
  • 5) Sokol RJ, Hewitt S, Stamps BK, Hitchen PA. Autoimmune haemolysis in childhood andadolescence. Acta Haematol. 1984;72(4):245-57.
  • 6) Zuelzer WW, Mastrangelo R, Stulberg CSPoulik MD, Page RH, Thompson RI.Autoimmune hemolytic anemia. Natural history and viral-immunologic interactions in childhood. Am J Med. 1970;49(1):80-93.
  • 7) Blackall DP. Warm-reactive autoantibodies in pediatric patients: clinical and serologic correlations. J Pediatr Hematol Oncol.2007;29(11):792-6.
  • 8) Naithani R, Agrawal N, Mahapatra M,Kumar R, Pati HP, Choudhry VP.Autoimmune hemolytic anemia in children.Pediatr Hematol Oncol. 2007;24(4):309-15.
  • 9) Oliveira MC, Oliveira BM, Murao M, Vieira ZM, Gresta LT, Viana MB. Clinicalcourse of autoimmune hemolytic anemia:an observational study. J Pediatr. (Rio J)2006;82(1):58-62.
  • 10) Vaglio S, Arista MC, Perrone MP, Tomei G,Testi AM, Coluzzi S, et al. Autoimmune hemolytic anemia in childhood: serologicfeatures in 100 cases. Transfusion. 2007;47(1):50-4.
  • 11) Evans RS, Takahashi K, Duane RT, Payne R,Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951;87(1):48-65
  • 12) Mathew P, Chen G, Wang W. Evans syndrome:results of a national survey. J Pediatr Hematol Oncol. 1997;19(5):433-7.
  • 13) Ng SC. Evans syndrome: a report on 12 patients. Clin Lab Haematol. 1992;14(3):189-93.
  • 14) Pui CH, Wilimas J, Wang W. Evans syndrome in chil18. Wang WC. Evans syndrome in childhood:pathophysiology, clinical course, and treatment.Am J Pediatr
  • 15) Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans' syndrome. Arch Dishild. 1997;77(3):245-8.
  • 16) Nelson DA, Davey FR. Erytrocytic disorders. In:Henry JB(Ed.). Clinical diagnosis and management by laboratory methods. 18th ed. Philadelphia: WB Saunders Co;1991;ch26,660-2
  • 17) Sarper N, Kılıc S, Zengin E, Gelen S A. Management of autoimmune hemolytic anemia in children and adolescents: A single center experience. Turk J Hematol 2011; 28: 198-205.
  • 18) Ware RE.Autoimmune hemolytic anemia In:Nathan DG,Orkin SH,Ginsburg D,Look AT eds.Nathan and Oski’s Hematology of Infancy and Chıldhood.6th ed.Philadelphia:W.B.Saunders;2003; 1:521-594.
  • 19) Pui, C., Wilimas, J. & Wang, W. Evans syndrome in childhood.The Journal of Pediatrics, 1980;97: 754–758.
  • 20) Wang, W. (1988) Evans syndrome in childhood: pathophysiology,clinical course, and treatment. The American Journal of PediatricHematology/Oncology, 10, 330–338.
  • 21) Mathew, P., Chen, G. & Wang, W. Evans Syndrome: results of anational survey. Journal of Pediatric Hematology/Oncology, 1997;19: 433–437.
  • 22) Savasan, S, Warrier, I. & Ravindranath, Y. The spectrumof Evans’ syndrome. Archives of Disease in Childhood, 1997;77: 245–248.
  • 23)Teachey, D.T., Manno, C.S., Axsom, K.M., Andrews, T., Choi, J.K.,Greenbaum, B.H., et all Unmasking Evans syndrome: T-cell phenotypeand apoptotic response reveal autoimmune lymphoproliferativesyndrome (ALPS). Blood, 2005;105: 2443–244
There are 23 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

Halil Çelik 0000-0002-2022-2909

Canan Albayrak This is me 0000-0002-9912-9626

Davut Albayrak 0000-0002-7947-3817

Publication Date April 30, 2020
Submission Date October 14, 2019
Published in Issue Year 2020 Volume: 3 Issue: 1

Cite

Vancouver Çelik H, Albayrak C, Albayrak D. Otoimmun Hemolitik Anemi ve Evans Sendromlu Çocuk Hastalarda Klinik Özelliklerin Değerlendirilmesi. MRR. 2020;3(1):16-21.