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Clinical and laboratory aspects in 80 subjects with antiphospholipid syndrome (APS)
Suppl. XIII, 1991
ABSTRACTS
67
133 FACTOR VIII INHIBITOR IN POST-PARTUM.TREATMENT WITH HIGHLDOSE INTRAVENOUS IMMUNOGLOBULIN.V.Trapani Lombardo, C.Latella, A. Piromalli. Centro Emofilia della Divisione di Ematologia OO.RR.-U.S.L. 31 Reggio Calabria Italy. Antibodies to factor VIII occur as autoantibodies in nonhemophilic patients. Inhibitor to factor VIII may appear , infact, during immune disease, malignancy, or during insulin-dependent diabetes or in asociation with drug assumption or in post-partum.These inhibitors, in nonhemophilic patients, may disappear spontaneously, but often are long lasting and are associated with severe bleeding complications. We report a case with factor VIII inhibitor in a post-par-turn patient, with severe spontaneous haematoma and petechiae. Coagulation test showed : factor VIII not dosable, factor VIIIc inhibitor 22 U.B.. Treatment with cortcosteroids (Deflazacort 2mg/kg/die) was started and gave partial remission (Inhibitor 4.2 U.B., factor VIII not dosable). Treatment with high dose of immunoglobulin (400mg/kg/5 day, and then maintenance therapy for six months) obtained a rapid clinic remission with complete disappearance of inhibitor after ten months; we observed a dramatic decrease of inhibitor (1.2 U.B, factor VIII 41%) after two months of treatment.We evaluate in vitro a neutralisation capacity of Ig son the anti factor VIII activity.
134 LiiniCA~
FINDLABORATORY ASPECTS IN 80 SUBJECl'sWITk ANTIPHOSPHOLIPID SYNOROM
CAPS).
L_Q&m, C, Ettorre, !I,&, Eillibertr, 6, Hicco,N, Ciavareila, Servlzlo di Coagulazione - CentroEmoiiira, Policilnico-Universita, Bari, Since
1383we navestuaied80 subjects (61 females and i8 maies), mean age 27 (range 3-71)
with APS, 53 individuals iC;6%) presented a history of iaiopathic thrombosis and/or repeated abortions, while 27 (34%) seemed to or asymptomatic and considered on the basis of prolonged APTT (ratio>1,2)uncorrecteu by mixing I:1 of tested plasma with pooled normai plasma, In all the subjects the presence of lupus anticoagulant (LA, was also checked by determinatjon ot Kaolin Clotting Time and Tissue Thrombopiastin Inhibition Test, considered positive at ratio value )1,2, Anticardiolipin antibodies (CICA) were detected in 70 subjects by ELI% cpathological value :*7U), 6'3 individuals (86%) were affected by primary MS and I1 (Id%) by APS associated with other diseases, of which 5 documented LES. In the group with primary APS recurrent abortions were prevalent 171%) in contront to the arterious il3X) and venous 110%) thrombosis, probably due to the prevalence of female population, In the group witn secondary APS no difference, in terms of prevalence, was observed regarding the pathological events, In both groups of inuividuals with either symptomatic or asymptomatic APS, concerning the laboratory findings, the classic pattern LA+/ACA+was prevalent i>SOZJ if compared with those LA+/ACA- and LA-/fiCA+, Is the pattern LA+/ACA+ an useful marker in order to identify the asymptomatic individuals with BPS at risk of thrombosis ?
ABSTRACTS
67
133 FACTOR VIII INHIBITOR IN POST-PARTUM.TREATMENT WITH HIGHLDOSE INTRAVENOUS IMMUNOGLOBULIN.V.Trapani Lombardo, C.Latella, A. Piromalli. Centro Emofilia della Divisione di Ematologia OO.RR.-U.S.L. 31 Reggio Calabria Italy. Antibodies to factor VIII occur as autoantibodies in nonhemophilic patients. Inhibitor to factor VIII may appear , infact, during immune disease, malignancy, or during insulin-dependent diabetes or in asociation with drug assumption or in post-partum.These inhibitors, in nonhemophilic patients, may disappear spontaneously, but often are long lasting and are associated with severe bleeding complications. We report a case with factor VIII inhibitor in a post-par-turn patient, with severe spontaneous haematoma and petechiae. Coagulation test showed : factor VIII not dosable, factor VIIIc inhibitor 22 U.B.. Treatment with cortcosteroids (Deflazacort 2mg/kg/die) was started and gave partial remission (Inhibitor 4.2 U.B., factor VIII not dosable). Treatment with high dose of immunoglobulin (400mg/kg/5 day, and then maintenance therapy for six months) obtained a rapid clinic remission with complete disappearance of inhibitor after ten months; we observed a dramatic decrease of inhibitor (1.2 U.B, factor VIII 41%) after two months of treatment.We evaluate in vitro a neutralisation capacity of Ig son the anti factor VIII activity.
134 LiiniCA~
FINDLABORATORY ASPECTS IN 80 SUBJECl'sWITk ANTIPHOSPHOLIPID SYNOROM
CAPS).
L_Q&m, C, Ettorre, !I,&, Eillibertr, 6, Hicco,N, Ciavareila, Servlzlo di Coagulazione - CentroEmoiiira, Policilnico-Universita, Bari, Since
1383we navestuaied80 subjects (61 females and i8 maies), mean age 27 (range 3-71)
with APS, 53 individuals iC;6%) presented a history of iaiopathic thrombosis and/or repeated abortions, while 27 (34%) seemed to or asymptomatic and considered on the basis of prolonged APTT (ratio>1,2)uncorrecteu by mixing I:1 of tested plasma with pooled normai plasma, In all the subjects the presence of lupus anticoagulant (LA, was also checked by determinatjon ot Kaolin Clotting Time and Tissue Thrombopiastin Inhibition Test, considered positive at ratio value )1,2, Anticardiolipin antibodies (CICA) were detected in 70 subjects by ELI% cpathological value :*7U), 6'3 individuals (86%) were affected by primary MS and I1 (Id%) by APS associated with other diseases, of which 5 documented LES. In the group with primary APS recurrent abortions were prevalent 171%) in contront to the arterious il3X) and venous 110%) thrombosis, probably due to the prevalence of female population, In the group witn secondary APS no difference, in terms of prevalence, was observed regarding the pathological events, In both groups of inuividuals with either symptomatic or asymptomatic APS, concerning the laboratory findings, the classic pattern LA+/ACA+was prevalent i>SOZJ if compared with those LA+/ACA- and LA-/fiCA+, Is the pattern LA+/ACA+ an useful marker in order to identify the asymptomatic individuals with BPS at risk of thrombosis ?