Author/Editor		Rožman, Primož
Title		Fetomaternalna aloimunska trombocitopenija
Translated title		Fetomaternal alloimmune thrombocytopenia
Type		članek
Source		Med Razgl
Vol. and No.		Letnik 39, št. Suppl 7
Publication year		2000
Volume		str. 23-30
Language		slo
Abstract		Fetomaternal alloimunne thrombocytopenia (FMAIT) is a disease of the fetus and neonate caused by anti-HPA antibodies produced by pregnant women in the course of an immune response to fetal HPA alloantigens inherited from the father. In whites, it is usually caused by the anti-HPA-1a (in 80-90% of cases), less frequently by anti-HPA-Sb (in 5-15% cases) and only rarely by other antibodies. Incidence of FMAIT is around 1.5 in 1,000 births and it is responsible for around 10% of all neonatal thrombocytopenias with a platelet count below 100x109/1. The fetus or the neonate with FMAIT displays mucosal and skin bleeding whereas in severe cases, intracranial haemorhages are observed with consequent neurological damage or death. The best treatment of FMAIT is a transfusion of HPA-compatible platelets. The use of i. v. immunoglobulins for pregnant women is less effective. If the fetus shows serious tendency of bleeding due to thrombocytopenia, regular weekly cordocenteses with intrauterine platelet transfusions are indicated. This treatment, however, still bears an approximately 2% mortality rate. Since the prevention of FMAIT is easier than its treatment and since the incidence of HPA-1a alloimmunizations in whites justifies the introduction of preventive screening tests, the question of a prenatal program for the detection of pregnancies at risk for FMAIT was raised, as in the case of routine testing for the prevention of haemolitic disease of the newborn.
Summary		Fetomaternalna aloimunska trombocitopenija (FMAIT) je bolezen ploda in novorojenca, ki nastane zaradi protiteles anti-HPA, ki jih tvori nosečnica v odgovor na trombocitne antigene, ki jih plod podeduje od očeta. Ponavadi jo povzročijo protitelesa anti-HPA-1a (80-90% primerov), redkeje anti-HPA-Sb (5-15 %) in le občasno protitelesa drugih specifičnosti. Incidenca fetomaternalne aloimunske trombocitopenije je okrog 1,5 na 1.000 porodov in je odgovorna za okrog 10% vseh neonatalnih trombocitopenij, pri katerih pade število trombocitov pod 100 x 10(9)/l. Plod ali novorojenček s fetomaternalno aloimunsko trombocitopenijo krvavita v sluznice in kožo, v težjih primerih pa pride do intrakranialnih krvavitev z nevrološkimi zapleti ali smrtjo. Fetomaternalno aloimunsko trombocitopenijo najbolj učinkovito zdravimo s transfuzijo skladnih trombocitov, medtem ko je preventivna uporaba steroidov in imunoglobulinov pri nosečnici manj uspešna. Če je zaradi trombocitopenije ogrožen že plod, so potrebne redne tedenske kordocenteze in znotrajmaternične transfuzije trombocitov, ki pa imajo okrog 2 % smrtnost. Ker je fetomaternalno aloimunsko trombocitopenijo laže preprečevati kot zdraviti in ker pojavnost aloimunizacij na HPA-1a pri belcih opravičuje presejalne teste, velja razmisliti o uvedbi preventivnih preiskav nosečnic, s katerimi bi določili rizične nosečnosti in jih pozorneje spremljali, podobno kot je to že v praksi pri predrojstveni zaščiti hemolitične bolezni novorojenca.
Descriptors		THROMBOCYTOPENIA PLATELET TRANSFUSION ANTIGENS, HUMAN PLATELET PLATELET GLYCOPROTEIN GPIB-IX COMPLEX PLATELET GLYCOPROTEIN GPIIB-IIIA COMPLEX