A rare condition: subgroup incompatibility due to anti E

Abstract

To the Editor While immune hydrops occurs mostly in relation with ABO and Rh incompatibility 95 97 it occurs rarely in relation with subgroup incompatibility 3 5 1 Subgroup incompatibilities lead to neonatal hemolytic anemia with varying degrees prolonged jaundice and rarely immune hydrops The most common subgroup incompatibilities which lead to blood incompatibility between the mother and the baby include C c E e antigens in the Rh system and K antigene in the Kell system 14 of the subgroup incompatibilities are related to anti E antibody 2 The clinical picture related to anti E mostly consists of mild moderate anemia and prolonged jaundice; hydrops fetalis is observed rarely 3 In the literature cases of hemolytic anemia related to anti K anti E anti C subgroup incompatibility have been rarely reported 4 5 6 7 8 9 In this letter we wished to report a rare case of hydrops fetalis which developed in the neonatal period as a result of anti E subgroup incompatibility Our patient was born by cesarean section at the 34th gestational week because of pleural fluid and fetal distress with a birth weight of 2200 g as the first live birth from the first pregnancy of a 26 year old mother G1A0P1 The patient who appeared pale and edematous was intubated in the delivery room and was internalized in the neonatal intensive care unit Diffuse edema was found on physical examination The apical heart beat was found to be 190 min and the blood pressure was found to be 70 40 mmHg The patient had hepatosplenomegaly and a 3 6 systolic murmur Neurologic examination revealed marked hypotonia and weak newborn reflexes The laboratory tests were as follows: the blood type of the baby: 0Rh the blood type of the mother: ARh direct Coombs test: negative Hemoglobin: 11 mg dL hematocrite: 34 lökosit: 9050 mm3 platelets: 17000 mm3 and reticulocyte: 8 Peripheral smear revealed 55 neutrophils 33 lymphocytes 12 normoblasts and fragmented erythrocytes and quartet platalet aggregations Pathological biochemical tests were as follows: total protein: 2 5 g dL albumin: 1 g dL Na: 128 mEq L total bilirubin: 4 mg dL On the postero anterior lung graphy fluid was found in bilateral fissures On thoracal ultrasonography USG pleural fluid 13 mm in the right and 11 mm in the left was observed On abdominal USG a small amount of freee fluid was found Cranial USG was normal Thoracentesis was performed and transudate fluid was evacuated Fluid was restricted because of hyponatremia and increased weight and phototherapy was started The results of the tests performed to determine non immune causes were as follows: TORCH S Parvovirüs B19 EBV IgM and reducing substance in urine Chromosome analysis metabolic screening tests hemoglobin electrophoresis glucose 6 P dehydrogenase echocardiography and electrocardiography were found to be normal Direct Coomb test was repeated because of exclusion of non immune causes of hydrops hemolysis findings and indirect hyperbilirubinemia and was found to be mildly positive Thereupon subgroup analysis was performed and anti E antigen incompatibility was found Intravenous immunoglobulin 1 g kg was administered to the patient On the 5th day ventilatory support and phototherapy were discontinued On the 13th day full enteral nutrition was started During the treatment albumin was administered for two times erythrocyte suspension was administered for one time and thrombocyte suspension was administered for two times On the 21st day the patient was discharged without any problem No problem was observed in the outpatient follow up Joy et al 3 found 283 anti E incompatibilities in the study they performed between 1959 and 2004 In 32 of these pregancies anemia in the newborn was found and hydrops was found only in one In the literature the rate of anti E positivity was found to be 0 12 in 43 000 women between 1994 and 1990 In only 5 of these findings of anemia developed in the baby 10 In the study performed recently by Karagol et al 11 in our country anti E incompatibility was found in 28 3 of 106 subgroup incompatibility cases but hydrops was not found in these cases In subgroup incompatibilities direct Coombs test is found positive with a rate of 33 A negative direct Coomb test is not a definite indicator of abscence of incompatibility Since subgroup incompatibility generally leads to mild anemia in the newborn baby it can be frequently overlooked However it should be kept in mind that subgroup incompatibility may lead to severe anemia and severe hyperbilirubinemia requiring exchange transfusion and rarely to a clinical picture of hydrops fetalis We wanted to remind of the necessity of further investigations to determine subgroup incompatibility in cases of hydrops fetalis Ad shy;dress for Cor shy;res shy;pon shy;den shy;ce: Evrim Kıray Baş MD Şişli Etfal Education and Research Hospital Neonatology Clinic İstanbul Turkey E mail: kiray_evrim@hotmail com Re shy;cei shy;ved: 09 18 2012 Ac shy;cep shy;ted: 12 17 2012 References 1 Stoll BJ Kleigman RM The fetus and the neonatal infant In: Behrman RE Kleigman RM Jenson HB eds Nelson textbook of pediatrics 17th ed Philadelphia: WB Saunders 2004: 592 607 2 Geifman Holtzman O Wojtowycz M Kosmas E Artal R Female alloimmunization with antibodies known to cause hemolytic disease Obstet Gynecol 1997; 89: 272 275 3 Joy SD Rossi KQ Krugh D O 39;Shaughnessy RW Management of pregnancies complicated by anti E alloimmunization Obstet Gynecol 2005 ; 105: 24 28 4 Aslan Y Erduran E Gedik Y Mocan H Yıldıran A Soylu H Kell C and E subgroup incompatıbilities in neonates with indirect hyperbilirubinemia Turkiye Klinikleri J Pediatr 1996; 5: 93 98 5 Özkaya H Bahar A Özkan A Kandemir F Göçmen İ Mete Z İndirekt hiperbilirubinemili yenidoğanlarda ABO Rh ve subgrup Kell c e uyuşmazlıkları Türk Ped Arş 2000; 35: 30 35 6 Özkaya H Kandemir F Süleymanoglu S Aydınöz S Ersen A Uğur E Ataş E Göçmen İ Anti E antibody related hemolytic disease of newborn: case report Nobel Medicus Dergisi 2006; 40: 571 574 7 Bolat F Bülbül A Uslu S Cömert S Can E Nuhoğlu A Anti Kell ve anti C alloimmünizasyonu: üç olgu sunumu The Medical Bulletin of Sişli Etfal Hospital 2009; 43: 142 145 8 Sarici SU Alpay F Yeşilkaya E Ozcan O Gökçay E Hemolytic disease of the newborn due to isoimmunization with anti E antibodies: a case report Turk J Pediatr 2002; 44: 248 250 9 Strohm PL Iams JD Kennedy MS Hemolytic disease of the newborn from anti E: A case report J Reprod Med 1988; 33: 404 406 10 Zipursky A Bowman JM Isoimmune hemolytic diseases In: Nathan DG Oski FA eds Hematology of infancy and childhood 4th ed Vol 1 Philadelphia: WB Saunders 1993: 44 73 11 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: 449 454

Keywords

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Nadir bir durum: anti E’ye bağlı subgrup uyuşmazlığı

Öz

İmmün hidrops en sık ABO ve Rh uyuşmazlığına (%95-97) bağlı oluşurken daha nadir olarak subgrup uyuşmazlığına (%3- 5) bağlı olarak da meydana gelir (1). Subgrup uyuşmazlıkları, değişik derecelerde yenidoğan hemolitik kansızlığına, uzamış sarılığa ve nadiren immün hidropsa neden olur. Anne ve bebek arasında kan uyuşmazlığına en sık neden olan subgrup uyuşmazlıkları ise Rh sisteminde bulunan C, c, E, e antijenleri ile Kell sisteminde bulunan K antijenidir. Subgrup uyuşmazlıklarının %14’ü Anti-E antikoruna bağlıdır (2). Anti-E nedeni ile ortaya çıkan klinik tablonun çoğunluğunu hafif-orta derecede kansızlık ve uzamış sarılık oluştururken hidrops fetalis tablosu nadirdir (3).

Anahtar Kelimeler

• Nadir
• anti E
• subgrup

Kaynakça

1. Stoll BJ, Kleigman RM. The fetus and the neonatal infant. In: Behrman RE, Kleigman RM, Jenson HB, (eds). Nelson textbook of pediatrics 17th ed. Philadelphia: WB Saunders, 2004: 592-607.
2. Geifman-Holtzman O, Wojtowycz M, Kosmas E, Artal R. Female alloimmunization with antibodies known to cause hemolytic disease. Obstet Gynecol 1997; 89: 272-5. 81 Türk Ped Arfl 2013; 48: 80-1 Turk Arch Ped 2013; 48: 80-1 Baş ve ark.
3. Nadir bir durum: anti-E’ye bağlı subgrup uyuşmazlığı / A rare condition: subgroup incompatibility due to anti-E