Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants

Direk Coombs’ testi pozitif hemolitik anemi ve dev hücreli hepatit birlikteliği çocukluk çağında patogenezi tam olarak aydınlatılmamış nadir bir durumdur. Bu hastaların bir kısmının immünosupresif tedaviye cevap veriyor olması otoimmün aktivasyon ya da kendi antijenlerine karşı tolerans kaybının patogenezde sorumlu olabileceğini düşündürmektedir. Bu makalede bu nadir duruma sahip üç hastanın başvuru özellikleri ve takibi sunulmuştur. Başvuru anındaki serum ferritin değerinin hastanın prognozunu belirlemede kullanılabileceği önerilmiştir.

Anahtar Kelime:

Konular: Hematoloji

Otoimmun hemolitik anemi ve dev hücreli hepatit birlikteliği olan üç olgunun sunumu

Öz:

Giant cell hepatitis associated with direct Coombs’ test-positive hemolytic anemia is a rare condition of childhood and the pathogenesis remains unclear. An autoimmune activation and loss of self-tolerance in these patients may be the underlying pathology related to the response of some of the patients to immunosuppressive treatment. Herein, we report the clinical presentation and course of three consecutive patients with this rare condition. We conclude that serum ferritin at diagnosis may be used for prediction of the outcome.

Anahtar Kelime:

Konular: Hematoloji

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık

1. Gorelik M, Debski R, Frangoul H. Autoimmune hemolytic anemia with giant cell hepatitis: case report and review of the literature. J Pediatr Hematol Oncol 2004;26:837-9.
2. Melendez HV, Rela M, Baker AJ, Ball C, Portmann B, Mieli-Vergani G, Heaton ND. Liver transplant for giant cell hepatitis with autoimmune hemolytic anemia. Arch Dis Child 1997;77:249-51.
3. Akyildiz M, Karasu Z, Arikan C, Nart D, Kilic M. Successful liver transplantation for giant cell hepatitis and Coombs-positive hemolytic anemia: a case report. Pediatr Transplant 2005;9:630-3.
4. Rovelli A, Corti P, Beretta C, Bovo G, Conter V, Mieli- Vergani G. Alemtuzumab for giant cell hepatitis with autoimmune hemolytic anemia. J Pediatr Gastroenterol Nutr 2007;45:596-9.
5. Miloh T, Manwani D, Morotti R, Sukru E, Shneider B, Kerkar N. Giant cell hepatitis and autoimmune hemolytic anemia successfully treated with rituximab. J Pediatr Gastroenterol Nutr 2007;44:634-6.
6. Perez-Atayade AR, Sirlin SM, Jonas M. Coombs-positive autoimmune hemolytic anemia and post-infantile giant cell hepatitis in children. Pediatr Pathol 1994;14:69-77.
7. Hartman C, Berkowitz D, Brik R, Arad A, Elhasid R, Shamir R. Giant cell hepatitis with autoimmune hemolytic anemia and hemophagocytosis. J Pediatr Gastroenterol Nutr 2001;32:330-4.
8. Kehrli ME Jr, Goff JP, Stevens MG, Boone TC. Coombs positive giant cell hepatitis—a new feature of Evans’ syndrome. Arch Dis Child 1998;78:397-8.
9. Weinstein T, Valderrama E, Pettei M, Levine J. Early steroid therapy for the treatment of giant cell hepatitis with autoimmune hemolytic anemia. J Pediatr Gastroenterol Nutr 1993;17:313-6.
10. Imgrueth M, Wagner HP, Pipczynski-Suter K, Beguin L, Wyss M, Pfister AM, Stock HK, Oetliker O.Plasma exchange: an important part of the therapeutic procedure in a small child with autoimmune hemolytic anemia. Acta Paediatr Scand 1986;75:1037-41.
11. Kashyap R, Sarangi JN, Choudhry VP. Autoimmune hemolytic anemia in an infant with giant cell hepatitis. Am J Hematol 2006;81:199-201.
12. Choulot JJ, Parent Y, Etcharry F, Saint-Martin J, Mensire A. Giant cell hepatitis and autoimmune hemolytic anemia: efficacy of splenectomy on hemolysis. Arch Pediatr 1996;3:789-91.
13. Vajro P, Migliaro F, Ruggeri C, Di Cosmo N, Crispino G, Caropreso M, Vecchione R. Life saving cyclophosphamide treatment in a girl with giant cell hepatitis and autoimmune haemolytic anaemia: case report and upto- date on therapeutical options. Dig Liver Dis 2005;38:846-50.
14. Bernard O, Hadchouel M, Scotto J, Odièvre M, Alagille D. Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. J Pediatr 1981;99: 704-11.
15. Brichard B, Sokal E, Gosseye S, Buts JP, Gadisseux JF, Cornu G. Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy. Eur J Pediatr 1991;150:314-7.
16. Camarero Salcés C, Vasconez Muñoz F, San José Hernando D, Morales P, Suárez Cortina L, Parra Vázquez C, Escobar Castro H, Muñoz Villa A. Autoimmune hemolytic anemia associated with severe giant cell hepatitis. An Esp Pediatr 1991;34:149-51.
17. Pappo O, Yunis E, Jordan JA, Jaffe R, Mateo R, Fung J, Demetris AJ. Recurrent and de novo giant cell hepatitis after orthotopic liver transplantation. Am J Surg Pathol 1994;18:804-13.

APA	Unal S, KUŞKONMAZ B, Balamtekin N, Baysoy G, ELMAS S, ORHAN D, KALE G, Yüce A, Gürakan F, GÜMRÜK F, ÇETIN M (2010). Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. , 308 - 313.
Chicago	Unal Sule,KUŞKONMAZ BÜLENT BARIŞ,Balamtekin Necati,Baysoy Gokhan,ELMAS Selin Aytaç,ORHAN Diclehan,KALE Gülsev,Yüce Aysel,Gürakan Figen,GÜMRÜK FATMA,ÇETIN MUALLA Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. (2010): 308 - 313.
MLA	Unal Sule,KUŞKONMAZ BÜLENT BARIŞ,Balamtekin Necati,Baysoy Gokhan,ELMAS Selin Aytaç,ORHAN Diclehan,KALE Gülsev,Yüce Aysel,Gürakan Figen,GÜMRÜK FATMA,ÇETIN MUALLA Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. , 2010, ss.308 - 313.
AMA	Unal S,KUŞKONMAZ B,Balamtekin N,Baysoy G,ELMAS S,ORHAN D,KALE G,Yüce A,Gürakan F,GÜMRÜK F,ÇETIN M Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. . 2010; 308 - 313.
Vancouver	Unal S,KUŞKONMAZ B,Balamtekin N,Baysoy G,ELMAS S,ORHAN D,KALE G,Yüce A,Gürakan F,GÜMRÜK F,ÇETIN M Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. . 2010; 308 - 313.
IEEE	Unal S,KUŞKONMAZ B,Balamtekin N,Baysoy G,ELMAS S,ORHAN D,KALE G,Yüce A,Gürakan F,GÜMRÜK F,ÇETIN M "Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants." , ss.308 - 313, 2010.
ISNAD	Unal, Sule vd. "Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants". (2010), 308-313.

APA	Unal S, KUŞKONMAZ B, Balamtekin N, Baysoy G, ELMAS S, ORHAN D, KALE G, Yüce A, Gürakan F, GÜMRÜK F, ÇETIN M (2010). Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. Turkish Journal of Hematology, 27(4), 308 - 313.
Chicago	Unal Sule,KUŞKONMAZ BÜLENT BARIŞ,Balamtekin Necati,Baysoy Gokhan,ELMAS Selin Aytaç,ORHAN Diclehan,KALE Gülsev,Yüce Aysel,Gürakan Figen,GÜMRÜK FATMA,ÇETIN MUALLA Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. Turkish Journal of Hematology 27, no.4 (2010): 308 - 313.
MLA	Unal Sule,KUŞKONMAZ BÜLENT BARIŞ,Balamtekin Necati,Baysoy Gokhan,ELMAS Selin Aytaç,ORHAN Diclehan,KALE Gülsev,Yüce Aysel,Gürakan Figen,GÜMRÜK FATMA,ÇETIN MUALLA Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. Turkish Journal of Hematology, vol.27, no.4, 2010, ss.308 - 313.
AMA	Unal S,KUŞKONMAZ B,Balamtekin N,Baysoy G,ELMAS S,ORHAN D,KALE G,Yüce A,Gürakan F,GÜMRÜK F,ÇETIN M Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. Turkish Journal of Hematology. 2010; 27(4): 308 - 313.
Vancouver	Unal S,KUŞKONMAZ B,Balamtekin N,Baysoy G,ELMAS S,ORHAN D,KALE G,Yüce A,Gürakan F,GÜMRÜK F,ÇETIN M Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants. Turkish Journal of Hematology. 2010; 27(4): 308 - 313.
IEEE	Unal S,KUŞKONMAZ B,Balamtekin N,Baysoy G,ELMAS S,ORHAN D,KALE G,Yüce A,Gürakan F,GÜMRÜK F,ÇETIN M "Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants." Turkish Journal of Hematology, 27, ss.308 - 313, 2010.
ISNAD	Unal, Sule vd. "Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants". Turkish Journal of Hematology 27/4 (2010), 308-313.