Hüsniye YÜCEL, İstemi Han ÇELİK, Ayşen Sumru KAVURT, Beyza ÖZCAN, Semih SANDAL, Ahmet Yağmur BAŞ, Nihal DEMİREL

Bir Yenidoğanda Tekrarlayan Intravenöz Immünoglobulin ve Eritrosit Transfüzyonu ile Tedavi Edilen Anti-E, C ve Kell İzoimmünizasyonuna Bağlı Ciddi Hemolitik Hastalık

Yenidoğanın alloimmün hemolitik hastalığının (YHH) etiyolojisinde Rhesus D dışındaki antikorların önemi giderek artmaktadır. Anti E, c ve Kell antikorlarının birlikte olduğu YHH daha önce bildirilmemiştir. Bu olgu sunumunda ciddi hemolitik hastalık ve sarılık nedeni ile tekrarlayan intravenöz immunoglobulin (İVİG) ve eritrosit transfüzyonu ile tedavi edilen bir vakayı sunmak istedik. Erkek yenidoğana doğumdan sonra 2 ve 3. günde 1 gr/kg IVIG verildi. Eritrosit transfüzyonu 6. günde yapıldı. Postnatal 12. günde hemoliz bulgularının devam etmesi üzerine 3. doz İVİG 1 gr/kg dozunda verildi. Taburculuk sonrası 3. haftada hemoliz bulguları saptanmadı. Sonuç olarak hemoliz, anemi ve sarılık saptanan yenidoğanlarda nadir olsa da birden fazla subgrup uyuşmazlığının olması akla gelmelidir. Fototerapi, İVİG ve eritrosit transfüzyonu tedavi seçenekleri arasındadır.

Anahtar Kelimeler:

Yenidoğanda izoimmünizasyon, anti-E, anti-c, anti-Kell, İVİG

A Neonate with Severe Hemolytic Disease Treated With Repeated Doses of Intravenous Immunoglobulin and Erythrocyte Transfusion Due to Anti-E, C and Kell Isoimmunization

Maternal Ig-G type blood group antibodies other than Rhesus D (RhD) are an increasingly significant cause of alloimmune hemolytic disease of the newborn (HDN). Anti-E, c and Kell associated HDN has not been reported in the literature. We report first case report of this combination with severe hemolytic anemia and hyperbilirubinemia requiring repeated doses of intravenous immunoglobulin (IVIG) and erythrocyte transfusion. He received 1 g/kg IVIG therapy on postnatal day 2 and 3 because of hemolysis. Erythrocyte transfusion was performed on postnatal day 6. Third dose IVIG as 1 gr/kg was given because of ongoing hemolysis on postnatal day 12. Three weeks after discharge he had no hemolysis. In conclusion, isoimmunization due to combination of subgroup incompatibility such as anti-E, c and Kell should be considered in patients with hemolysis, anemia and jaundice although combination of these isoimmunization is rare. Phototherapy, IVIG and erythrocyte transfusion are treatment options.

Keywords:

newborn isoimmunization, anti-E, anti-c, anti-Kell, IVIG,

PDF

___

Referans1. De Alarcon PA, EJ. W. Neonatal Hematology. New York: Cambridge University Press; 2005.
Referans2. Moise KJ, Jr. Non-anti-D antibodies in red-cell alloimmunization. European journal of obstetrics, gynecology, and reproductive biology 2000; 92: 75-81. Referans3. Dajak S, Stefanovic V, Capkun V. Severe hemolytic disease of fetus and newborn caused by red blood cell antibodies undetected at first-trimester screening (CME). Transfusion 2011; 51: 1380-8.
Referans4. Westhoff CM. The Rh blood group system in review: a new face for the next decade. Transfusion 2004; 44: 1663-73.
Referans5. Eder AF. Update on HDFN: new information on long-standing controversies. Immunohematology / American Red Cross 2006; 22: 188-95.
Referans6. Babinszki A, Berkowitz RL. Haemolytic disease of the newborn caused by anti-c, anti-E and anti-Fya antibodies: report of five cases. Prenatal diagnosis 1999; 19: 533-6.
Referans7. Bashawri LA. A Case of Haemolytic Disease of the Newborn Due to Maternal Anti-E and Anti-c. Bahrain Medical Bulletin 2007; 29:
Referans8. Farnault L, Garcia-Meric P, Cortey A, Arnaud F. [Fetomaternal anti-RH3, -4 (anti-E and anti-c) rhesus isoimmunization: a case report]. Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 2011; 18: 176-82.
Referans9. Gottstein R, Cooke RW. Systematic review of intravenous immunoglobulin in haemolytic disease of the newborn. Archives of disease in childhood Fetal and neonatal edition 2003; 88: F6-10.
Referans10. Demirel G, Akar M, Celik IH, Erdeve O, Uras N, Oguz SS, et al. Single versus multiple dose intravenous immunoglobulin in combination with LED phototherapy in the treatment of ABO hemolytic disease in neonates. International journal of hematology 2011; 93: 700-3.
Referans11. Onesimo R, Rizzo D, Ruggiero A, Valentini P. Intravenous Immunoglobulin therapy for anti-E hemolytic disease in the newborn. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstet 2010; 23: 1059-61.
Referans12. Karagol BS, Zenciroglu A, Okumus N, Karadag N, Dursun A, Hakan N. Hemolytic disease of the newborn caused by irregular blood subgroup (Kell, C, c, E, and e) incompatibilities: report of 106 cases at a tertiary-care centre. Am J Perinatol 2012; 29: 449-54.