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Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients

Mingot-Castellano, María E.a; Moret, Andrésb; de Cos, Carmenc; García-Candel, Faustinod; Garrido, Raquele; González-Porras, José R.f; López-Fernández, María F.g; Quintana, Laurah; Rodríguez-González, Ramóni; Marco, Pascualj

Blood Coagulation & Fibrinolysis: April 2019 - Volume 30 - Issue 3 - p 127–132
doi: 10.1097/MBC.0000000000000804

We hypothesized that inhibitor specificity may predict the outcome of antifactor VIII autoantibodies eradication treatment in acquired hemophilia A. Our objective was to analyze the association between factor VIII domains recognized by inhibitors and outcome of the immunosuppressive therapies (ISTs) in a prospective, observational study. 16 patients were recruited. Inhibitor specificities were assessed at diagnosis and throughout the study. Their association with IST outcome was addressed. First-line IST succeeded in 56% of patients. Inhibitors reacted mainly with light chain domains (69%) and/or the A2 domain (44%). 31% inhibitors recognized more than one domain. Significantly, the number of patients whose inhibitors recognized the light chain was significantly higher in the group of those who did not reach complete remission after first line IST when compared with those who did [6/7 (85.7%) vs. 4/9 (44.4%), P < 0.05]. Therefore, inhibitor specificity could predict the success of IST in acquired hemophilia A.

aHospital Regional Universitario de Málaga, Málaga

bInstituto de Investigación Sanitaria La Fe, Valencia

cHospital Universitario Puerta del Mar, Cádiz

dHospital Clínico Universitario Virgen de la Arrixaca, Murcia

eHospital de Puerto Real, Cádiz

fHospital Universitario de Salamanca-IBSAL, Salamanca

gComplejo Hospitalario Universitario A Coruña, A Coruña

hHospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria

iHospital Severo Ochoa, Leganés, Madrid

jHospital General Universitario, Alicante, Spain

Correspondence to María E. Mingot-Castellano, Hospital Regional Universitario de Málaga, Málaga, Spain E-mail:

Received 7 June, 2018

Revised 19 October, 2018

Accepted 14 January, 2019

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