A case of FXIIi (fibrin stabilizing factor, Laki-Lóránd factor) deficiency
Boda, Z.; Muszbek, L.; Hársfalvi, J.; Tóth, A.; Pfliegler, G.; Tornai, I.; Rák, K.
Orvosi Hetilap 129(41): 2199-2202
1988
ISSN/ISBN: 0030-6002 PMID: 3054695 Document Number: 320190
The history of a 34-years-old woman is described as the first one of congenital FXIII deficiency (type I) in Hungary. The subunit A of FXIII was totally absent in the patient's plasma and platelets and considerably reduced in monocytes (belwo 5%). The plasmatic level of subunit B was 25% (markedly reduced). The bleeding tendency was accompanied by habitual abortions. Laboratory diagnosis of congenital FXIII deficiency was discussed in details. According to the patient's family study the inhertiency of congenital FXIII deficiency is autosomal recessive.