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Pregnancy complications in acquired thrombotic thrombocytopenic purpura: a case-control study.

Ferrari B, Maino A, Lotta LA, Artoni A, Pontiggia S, Trisolini SM, Malato A, Rosendaal FR, Peyvandi F - Orphanet J Rare Dis (2014)

Bottom Line: However, the exact entity of these risks and their causes are unknown.In the cases, ADAMTS13 activity levels in the first trimester were moderately-to-severely reduced (median levels <3% in gravidic TTP and median levels 20% [range 14-40%] in the women with miscarriage) and anti-ADAMTS13 antibodies were invariably present, while in the control group ADAMTS13 activity levels were normal (median 90%, range 40-129%), with absence of detectable anti-ADAMTS13 antibodies.ADAMTS13 activity evaluation and detection of anti-ADAMTS13 antibody could help to predict the risk of complications in pregnant women with a history of acquired TTP.

View Article: PubMed Central - PubMed

Affiliation: Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, Angelo Bianchi Bonomi Hemophilia and Thrombosis Centre, Milan, Italy. barbara.ferrari@policlinico.mi.it.

ABSTRACT

Background: Pregnant women with a history of acquired thrombotic thrombocytopenic purpura (TTP) are considered at risk for disease recurrence and might be at risk for miscarriage, similar to other autoimmune disorders. However, the exact entity of these risks and their causes are unknown. The aim of this study was to evaluate risk factors associated with adverse pregnancy outcome, in terms of both gravidic TTP and miscarriage, in women affected by previous acquired TTP.

Methods: We conducted a nested case-control study in women with a history of acquired TTP enrolled in the Milan TTP registry from 1994 to October 2012, with strict inclusion criteria to reduce referral and selection bias.

Results: Fifteen out of 254 women with acquired TTP were included, namely four cases with gravidic TTP, five with miscarriage, and six controls with uncomplicated pregnancy. In the cases, ADAMTS13 activity levels in the first trimester were moderately-to-severely reduced (median levels <3% in gravidic TTP and median levels 20% [range 14-40%] in the women with miscarriage) and anti-ADAMTS13 antibodies were invariably present, while in the control group ADAMTS13 activity levels were normal (median 90%, range 40-129%), with absence of detectable anti-ADAMTS13 antibodies. Reduced levels of ADAMTS13 activity (<25%) in the first trimester were associated with an over 2.9-fold increased risk for gravidic TTP and with an over 1.2-fold increased risk for miscarriage (lower boundary of the confidence interval of the odds ratio). In addition, the presence of anti-ADAMTS13 antibodies during pregnancy was associated with an over 6.6-fold increased risk for gravidic TTP and with an over 4.1-fold increased risk for miscarriage.

Conclusions: ADAMTS13 activity evaluation and detection of anti-ADAMTS13 antibody could help to predict the risk of complications in pregnant women with a history of acquired TTP.

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Related in: MedlinePlus

ADAMTS13 activity levels in the first trimester according to pregnancy outcomes.Abbreviations: TTP, Thrombotic thrombocytopenic purpura. For women with multiple tests, mean values are reported.
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Fig2: ADAMTS13 activity levels in the first trimester according to pregnancy outcomes.Abbreviations: TTP, Thrombotic thrombocytopenic purpura. For women with multiple tests, mean values are reported.

Mentions: Gravidic TTP recurrences developed in the second or third trimester, while all five miscarriages occurred in the first trimester (i.e., by 13th +1 gestational week). ADAMTS13 activity levels in the first trimester were significantly reduced in the cases of gravidic TTP, with median levels <3%, while ADAMTS13 deficiency was less severe in the cases of miscarriage, with median levels of 20% (range 14-40%). Anti-ADAMTS13 antibodies resulted to be invariably present in either group of cases, with median anti-ADAMTS13 IgG levels of 4% (range 3–7.5%) in women with gravidic TTP and 2% (range <1.18-4.4%) in the miscarriage group. In the control group of women with uncomplicated pregnancies ADAMTS13 activity levels were normal in the first trimester (median 90%, range 40-129%) and remained above 39% until delivery, with absence of detectable anti-ADAMTS13 antibodies (Figure 2).Figure 2


Pregnancy complications in acquired thrombotic thrombocytopenic purpura: a case-control study.

Ferrari B, Maino A, Lotta LA, Artoni A, Pontiggia S, Trisolini SM, Malato A, Rosendaal FR, Peyvandi F - Orphanet J Rare Dis (2014)

ADAMTS13 activity levels in the first trimester according to pregnancy outcomes.Abbreviations: TTP, Thrombotic thrombocytopenic purpura. For women with multiple tests, mean values are reported.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4279798&req=5

Fig2: ADAMTS13 activity levels in the first trimester according to pregnancy outcomes.Abbreviations: TTP, Thrombotic thrombocytopenic purpura. For women with multiple tests, mean values are reported.
Mentions: Gravidic TTP recurrences developed in the second or third trimester, while all five miscarriages occurred in the first trimester (i.e., by 13th +1 gestational week). ADAMTS13 activity levels in the first trimester were significantly reduced in the cases of gravidic TTP, with median levels <3%, while ADAMTS13 deficiency was less severe in the cases of miscarriage, with median levels of 20% (range 14-40%). Anti-ADAMTS13 antibodies resulted to be invariably present in either group of cases, with median anti-ADAMTS13 IgG levels of 4% (range 3–7.5%) in women with gravidic TTP and 2% (range <1.18-4.4%) in the miscarriage group. In the control group of women with uncomplicated pregnancies ADAMTS13 activity levels were normal in the first trimester (median 90%, range 40-129%) and remained above 39% until delivery, with absence of detectable anti-ADAMTS13 antibodies (Figure 2).Figure 2

Bottom Line: However, the exact entity of these risks and their causes are unknown.In the cases, ADAMTS13 activity levels in the first trimester were moderately-to-severely reduced (median levels <3% in gravidic TTP and median levels 20% [range 14-40%] in the women with miscarriage) and anti-ADAMTS13 antibodies were invariably present, while in the control group ADAMTS13 activity levels were normal (median 90%, range 40-129%), with absence of detectable anti-ADAMTS13 antibodies.ADAMTS13 activity evaluation and detection of anti-ADAMTS13 antibody could help to predict the risk of complications in pregnant women with a history of acquired TTP.

View Article: PubMed Central - PubMed

Affiliation: Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, Angelo Bianchi Bonomi Hemophilia and Thrombosis Centre, Milan, Italy. barbara.ferrari@policlinico.mi.it.

ABSTRACT

Background: Pregnant women with a history of acquired thrombotic thrombocytopenic purpura (TTP) are considered at risk for disease recurrence and might be at risk for miscarriage, similar to other autoimmune disorders. However, the exact entity of these risks and their causes are unknown. The aim of this study was to evaluate risk factors associated with adverse pregnancy outcome, in terms of both gravidic TTP and miscarriage, in women affected by previous acquired TTP.

Methods: We conducted a nested case-control study in women with a history of acquired TTP enrolled in the Milan TTP registry from 1994 to October 2012, with strict inclusion criteria to reduce referral and selection bias.

Results: Fifteen out of 254 women with acquired TTP were included, namely four cases with gravidic TTP, five with miscarriage, and six controls with uncomplicated pregnancy. In the cases, ADAMTS13 activity levels in the first trimester were moderately-to-severely reduced (median levels <3% in gravidic TTP and median levels 20% [range 14-40%] in the women with miscarriage) and anti-ADAMTS13 antibodies were invariably present, while in the control group ADAMTS13 activity levels were normal (median 90%, range 40-129%), with absence of detectable anti-ADAMTS13 antibodies. Reduced levels of ADAMTS13 activity (<25%) in the first trimester were associated with an over 2.9-fold increased risk for gravidic TTP and with an over 1.2-fold increased risk for miscarriage (lower boundary of the confidence interval of the odds ratio). In addition, the presence of anti-ADAMTS13 antibodies during pregnancy was associated with an over 6.6-fold increased risk for gravidic TTP and with an over 4.1-fold increased risk for miscarriage.

Conclusions: ADAMTS13 activity evaluation and detection of anti-ADAMTS13 antibody could help to predict the risk of complications in pregnant women with a history of acquired TTP.

Show MeSH
Related in: MedlinePlus