PO0028 - Report of clinical case: Acquired hemophilia with a high-titer inhibitor and hemorrhagic diathesis.

Wednesday, July 13, 2022

1:00 PM – 2:00 PM

Location: ExCel Center, ICC Capital Suite Room 1

Presenting Author(s)

Raquel Santoja Rubio

Resident
Hospital Universitario Arnau de Vilanova.
Lleida, Catalonia, Spain

Co-Author(s)

Albert Tugues

Hematologist
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
Lleida, Catalonia, Spain
EV

Eva Vicente

Hematologist
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
Lleida, Catalonia, Spain
CM

Cristina Marzo

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
Lérida, Catalonia, Spain
PM

Paula Monteagudo

Internist
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
LA

Liz Amuh

Resident
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
CC

Carlos Chávez

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
MT

Montserrat Teixidó

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
AG

Antonio Garcia

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
AL

Armando Luaña

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
AF

Ainhara Ferrero

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
AR

Anaïs Ruiz

Resident
Hospital Universitari Arnau de Vilanova de Lleida, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain
TG

Tomas Garcia

Hematologyst
Hospital Universitari Arnau de Vilanova de Lleida, Departamento de Trombosis y Hemostasia, Servicio de Hematología y Hemoterapia.
lleida, Catalonia, Spain

Background: Acquired hemophilia is a rare bleeding disorder with an underestimated incidence due to the complexity of diagnosis. It`s characterized by the presence of autoantibodies against coagulation Factor VIII, which can trigger bleeding episodes. Most cases are of idiopathic etiology. It has a high morbi-mortality, hence the importance of establishing an early diagnosis and treatment.

Aims: Establish, according to current guidelines, individualized therapeutic management, hemostatic, immunosuppressive and immunomodulatory treatment, as well as its monitoring and follow-up of the case studied.

Methods: Clinical case study of a 71-year-old patient with active bleeding, factor VIII level less than 1% and inhibitor titer greater than 40 Bethesda Units (UB) at the time of diagnosis.

Results: Given the absence of hemorrhagic symptoms, treatment with corticosteroids mg/kg was started after diagnosis. The etiological study did not identify cause.
After 24 hours, the patient presented significant subcutaneous hematomas requiring transfusion of packed red blood cells (HHCC) and the addition of activated recombinant Factor VII (arFVII). Also, immunosuppressive treatment with immunoglobulins and Rituximab was started.
After one week, due to new onset of bruising and progressive anemia despite HHCC transfusion, with persistence of FVIII < 1% and inhibitor >40 UB, cyclophosphamide was added to the therapeutic regimen and arFVII was changed to activated prothrombin complex.
After three weeks of intensive treatment, the evolution was satisfactory with cessation of hemorrhagic symptoms, presenting figures of FVIII 7% and inhibitor of 9 UB.

Conclusion(s): Despite being a rare entity, it is important to know the differential diagnosis in a patient with bleeding diathesis and alteration in coagulation tests due to its high morbidity and mortality. It requires close monitoring in addition to intensive treatment, with the aim of controlling possible bleeding complications and reducing the autoantibody titer through different therapeutic schemes, trying to control the disease and achieve remission.