Abstract
Original language | English |
---|---|
Pages (from-to) | 47-55 |
Journal | Blood |
Volume | 120 |
Issue number | 1 |
DOIs | |
Publication status | Published - 5 Jul 2012 |
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In: Blood, Vol. 120, No. 1, 05.07.2012, p. 47-55.
Research output: Contribution to journal › Article › Academic › peer-review
TY - JOUR
T1 - Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)
AU - Collins, Peter
AU - Baudo, Francesco
AU - Knoebl, Paul
AU - Levesque, Herve
AU - Nemes, Laszlo
AU - Pellegrini, Fabio
AU - Huth-Kuehne, Angela
AU - Aspoeck, Gerold
AU - Heistinger, Max
AU - Knobl, Paul
AU - Makipernaa, Anne
AU - Andre, Helene
AU - Aouba, A
AU - Bellucci, Sylvia
AU - Beurrier, Philippe
AU - Borg, Jeanne Yvonne
AU - Darnige, Luc
AU - Devignes, Jean
AU - dOiron, Roseline
AU - Gautier, Philippe
AU - Gay, Valerie
AU - Girault, Stephane
AU - Gruel, Yves
AU - Guerin, Viviane
AU - Hezard, Nathalie
AU - Khellaf, Mehdi
AU - Koenig, Martial
AU - Lifermann, Francois
AU - Marlu, Raphael
AU - Ninet, J.
AU - Peynet, Jocelyne
AU - Quemeneur, Thomas
AU - Rothschild, Chantal
AU - Schleinitz, Nicolas
AU - Sigaud, Marianne
AU - Trouillier, Sebastien
AU - Voisin, Sophie
AU - Giebl, Andreas
AU - Holstein, Katharina
AU - Huth-Kuhne, Angela
AU - Loreth, Ralph M.
AU - Steigerwald, Udo
AU - Tiede, Andreas
AU - Theodossiades, George
AU - Radvanyi, Gaspar
AU - Schlammadinger, Agota
AU - Barillari, Giovanni
AU - Pasca, Samantha
AU - Caimi, T.
AU - Contino, L.
AU - D'Angelo, Armando
AU - Crippa, Luciano
AU - Fattorini, Annalisa
AU - Di Minno, Giovanni
AU - Cerbone, Anna Maria
AU - Di Minno, Matteo Nicola Dario
AU - D'inca, Marco
AU - Falanga, Anna
AU - Maggioni, Anna
AU - Lerede, Teresa
AU - Franchini, Massimo
AU - Gaidano, Gianluca
AU - De Paoli, Lorenzo
AU - Gamba, Gabriella
AU - Ghirardi, Raffaele
AU - Girotto, Mauro
AU - Tasca, Delios
AU - Grandone, Elvira
AU - Tiscia, Giovanni
AU - Imberti, Davide
AU - Iorio, Alfonso
AU - Landolfi, Raffaele
AU - Di Gennaro, Leonardo
AU - Novarese, Linda
AU - Mariani, Guglielmo
AU - Lapecorella, Mario
AU - Marietta, Marco
AU - Pedrazzi, Paola
AU - Mazzucconi, Maria Gabriella
AU - Santoro, Cristina
AU - Morfini, Massimo
AU - Linari, Silvia
AU - Moratelli, Stefano
AU - Paolini, Rossella
AU - Piseddu, Gavino
AU - Poggio, Renzo
AU - Pogliani, Enrico
AU - Carpenedo, Monica
AU - Remiddi, Chiara
AU - Santagostino, Elena
AU - Mancuso, Maria Elisa
AU - Santoro, Rita
AU - Papaleo, Giuseppina
AU - Schinco, Piercarla
AU - Borchiellini, Alessandra
AU - Valeri, Federica
AU - Scortechini, Anna Rita
AU - Siragusa, Sergio
AU - Sottilotta, Gianluca
AU - Squizzato, Alessandro
AU - Tagariello, Giuseppe
AU - Sartori, Roberto
AU - Tagliaferri, Anna Rita
AU - Di Perna, Caterina
AU - Rivolta, Gianna Franca
AU - Testa, Sophie
AU - Paoletti, Oriana
AU - Toschi, Vincenzo
AU - Zanon, Ezio
AU - Brandolin, Barbara
AU - Hamulyak, Karly
AU - Kamphuisen, Pieter
AU - Laros-van Gorkom, Britta
AU - Leebeek, Frank W.G.
AU - Marten, Nijziel
AU - Novakova, Irena
AU - Schutgens, Roger
AU - van der Linden, P.W.G
AU - van Esser, Joost
AU - van der Meer, J.
AU - Ypma, Paula
AU - Campos, Manuel
AU - Aguilar, Carlos
AU - Altisent, Carmen
AU - Bermejo, Nuria
AU - Del Campo, Raquel
AU - Ferreiro Arguelles, M.
AU - Gonzalez Boullosa, Rosario
AU - Gutierrez Pimentel, Maria Jose
AU - Jimenez-Yuste, Victor
AU - Jose-Felix, Lucia
AU - Marco, Pascual
AU - Mingot, Maria Eva
AU - Perez Garrido, Rosario
AU - Perez Gonzale, Noelia z
AU - Prieto Garcia, Manuel
AU - Rodriguez-Huerta, Ana Maria
AU - Maranon, HGUG
AU - Sedano, Carmen
AU - Tolosa Munoz, Alexandra
AU - Baghaei, Fariba
AU - Tengborn, Lilian
AU - Boehlen, Francoise
AU - Korte, Wolfgang
AU - Chowdary, Pratima
AU - Evans, Gillian
AU - Pavord, Suzanne
AU - Rangarajan, Savita
AU - Wilde, Jonathan
PY - 2012/7/5
Y1 - 2012/7/5
N2 - Acquired hemophilia A (AHA) is an autoimmune disease caused by an autoantibody to factor VIII. Patients are at risk of severe and fatal hemorrhage until the inhibitor is eradicated, and guidelines recommend immunosuppression as soon as the diagnosis has been made. The optimal immunosuppressive regimen is unclear; therefore, data from 331 patients entered into the prospective EACH2 registry were analyzed. Steroids combined with cyclophosphamide resulted in more stable complete remission (70%), defined as inhibitor undetectable, factor VIII more than 70 IU/dL and immunosuppression stopped, than steroids alone (48%) or rituximab-based regimens (59%). Propensity score-matched analysis controlling for age, sex, factor VIII level, inhibitor titer, and underlying etiology confirmed that stable remission was more likely with steroids and cyclophosphamide than steroids alone (odds ratio = 3.25; 95% CI, 1.51-6.96; P <.003). The median time to complete remission was approximately 5 weeks for steroids with or without cyclo-phosphamide; rituximab-based regimens required approximately twice as long. Immunoglobulin administration did not improve outcome. Second-line therapy was successful in approximately 60% of cases that failed first- line therapy. Outcome was not affected by the choice of first-line therapy. The likelihood of achieving stable remission was not affected by underlying etiology but was influenced by the presenting inhibitor titer and FVIII level. (Blood. 2012;120(1):47-55)
AB - Acquired hemophilia A (AHA) is an autoimmune disease caused by an autoantibody to factor VIII. Patients are at risk of severe and fatal hemorrhage until the inhibitor is eradicated, and guidelines recommend immunosuppression as soon as the diagnosis has been made. The optimal immunosuppressive regimen is unclear; therefore, data from 331 patients entered into the prospective EACH2 registry were analyzed. Steroids combined with cyclophosphamide resulted in more stable complete remission (70%), defined as inhibitor undetectable, factor VIII more than 70 IU/dL and immunosuppression stopped, than steroids alone (48%) or rituximab-based regimens (59%). Propensity score-matched analysis controlling for age, sex, factor VIII level, inhibitor titer, and underlying etiology confirmed that stable remission was more likely with steroids and cyclophosphamide than steroids alone (odds ratio = 3.25; 95% CI, 1.51-6.96; P <.003). The median time to complete remission was approximately 5 weeks for steroids with or without cyclo-phosphamide; rituximab-based regimens required approximately twice as long. Immunoglobulin administration did not improve outcome. Second-line therapy was successful in approximately 60% of cases that failed first- line therapy. Outcome was not affected by the choice of first-line therapy. The likelihood of achieving stable remission was not affected by underlying etiology but was influenced by the presenting inhibitor titer and FVIII level. (Blood. 2012;120(1):47-55)
U2 - 10.1182/blood-2012-02-409185
DO - 10.1182/blood-2012-02-409185
M3 - Article
C2 - 22517903
SN - 0006-4971
VL - 120
SP - 47
EP - 55
JO - Blood
JF - Blood
IS - 1
ER -