Author/Editor     Černelč, Peter; Tomažič, Janez
Title     Zdravljenje krvavitev in protiteles za FVIII pri bolnikih s pridobljeno hemofilijo A
Translated title     Treatment of bleeding and factor VIII inhibitors in acquired hemophilia A patients
Type     članek
Source     Zdrav Vestn
Vol. and No.     Letnik 73, št. Suppl 1
Publication year     2004
Volume     str. I-159-61
Language     slo
Abstract     Background. Bleeding in acquired hemophilia A patients with FVIll autoantibodies is often severe. The authors describe a successfuly acquired hemophilia A treatment with Feiba, glucocorticoids and cyclophosphamide in three patients. Results. In a 66-year patient with chest hematoma and m. iliopsoas hematoma, APTT 50.1 s, FVIII 0.05 IU/mL and inhibitors FVIII (anti-FVIIl) 22 BU/ml were found. After treatment of hemorrhages with Feiba, dexamethasone and cyclophosphamide, FVlll increased to 0.54 IU/mL and no inhibitors were found on day seventeen. In a 46year patient with advancedAIDS, multiple hematomas and liver cirrhosis after HCV infection, the platelet count was 92 X 10`/L, APTT 48.2 s, FVIII 0.05 IU/mL and anti-FVlll1.7BU/mL. The hemorrhages were treated with FEIBA and methylprednisolone therapy was started after 24 months. After 42 days FVIII increased to 0.56 IU/mL and no inhibitors were found. In a 69-year patient suffering from thyroiditis and idiopathic thrombocytopenic purpura, extensive gluteal and chest hematomas were detected during the maintenance therapy with methylprednisolone 16 mg/day. The thrombocyte count was normal but APTT 48.8 s, FVIII 0.01 IU/mL and anti-FVIII 7.0 BU/mL was found. FVIII activity were 2.72 IU/mL and no inhibitors was noted after 330-days of treatment with cyclophosphamide. Conclusions. The treatment with FEIBA was effective in stopping the hemorrhages. The glucocorticoids were efficient in prompt decreasing of the inhibitor titer whereas the effect of cyclophosphamide was retarded.
Summary     Izhodišča. Pri pridobljeni hemofiliji A zaradi protiteles za FVIII bolnika pogosto ogrožajo obsežne krvavitve. Prikazujemo tri bolnike s hudo obliko pridobljene hemofilije A, kjer smo krvavitve uspešno zdravili s FEIBO, protitelesa pa z glukokortikoidi in ciklofosfamidom. Rezultati. Pri 66-letnem bolniku s hematomom v steni prsnega koša in krvavitvijo v m. iliopsoas smo ugotovili: delni tromboplastinski čas (DTČ) 50,1 s, FVIII 0,05 E/ml in titer protiteles za FVII1 22 Be (Bethesda enot)/ml Po zdravljenju krvavitev s FEIBO, protiteles pa z deksametazonom in ciklofosfamidom, je porastel FVlll na 0,54 E/ml, protitelesa pa so izginila 17. dan. Pri 46-letnem bolniku z napredovalim aidsom in številnimi hematomi v koži, jetrno cirozo po hepatitisu C je bilo število trombocitov 92 x 10 9/L, DTČ 48,2 s, FVlll 0,05 E/ ml in titer protiteles za FVlll 1, 7 Be/ml. Po zdravljenju krvavitev s Feibo, smo po 14 mesecih začeli zdraviti z metilprednizonom. Po 42 dneh je porastel FVIII na 0,56E/ml, protitelesa pa so izginila. Pri 69-letni bolnici s tiroiditisom in idiopatično trombocitopenično purpuro smo ugotovili hematome v koži prsnega koša in glutealno med zdravljenjem z metilprednizonom 16 mg/dan. Število trombocitov je bilo normalno, DTČ 48, 8 s, FVIII 0, 01 E/ml in titer protiteles FVlll 7, 0 Be/ml Po 330-dnevnem zdravljenju s ciklofosfamidom je porastel FVIII na 1,72 E/ml, protitelesa so izginila. Zaključki. Zdravljenje krvavitev s FEIBO je bilo učinkovito pri vseh treh bolnikih. Zdravljenje protiteles z glukokortikoidi in ciklofosfamidom je bilo hitro učinkovito, medtem ko se je učinek ciklofosfamida pojavil kasneje.
Descriptors     HEMOPHILIA
FACTOR VIII
AUTOANTIBODIES
GLUCOCORTICOIDS, SYNTHETIC
CYCLOPHOSPHAMIDE
MIDDLE AGE
AGED
HEMORRHAGE