PB1235 - Acquired hemophilia A in very old patients: issues on therapy choice. A case report

Tuesday, June 27, 2023

18:30 – 19:30 ET

Room: Exhibition

Co-Author(s)

MG

Mariana Gradim

MD
Immunohemotherapy Department, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, Portugal
Porto, Porto, Portugal

Presenting Author(s)

Rui Alves

MD
Immunohemotherapy Department, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal
Lisboa, Lisboa, Portugal

Co-Author(s)

RQ

Rita Queirós

Resident doctor
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Póvoa de Lanhoso, Porto, Portugal
DL

Diana Leão

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal
LC

Lídia Costa

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal
SF

Susana Fernandes

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal
ML

Manuela Lopes

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal
MC

Manuela Carvalho

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal
CK

Carmo Koch

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal

Background: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII (FVIII). Severe bleeding occurs in 90% of patients with a high rate of morbimortality, especially in the elderly.

Aims: Case report of AHA at advanced age with management and therapeutic challenges.

Methods: Case report.

Results: A 95-year-old woman with multiple cardiovascular comorbidities, osteoporosis and chronic kidney disease was admitted to the emergency room of a tertiary hospital, after falling at home and presenting extensive bruising. She had large hematomas and hemoglobin 5.7g/dL, requiring transfusion support. CT-scan showed a huge deep intramuscular hematoma in left gluteal region(96x72x98mm). The coagulation screening revealed a prolonged activated partial thromboplastin time (aPTT) 133s, without correction in the mixing study. Further investigation revealed FVIII activity < 0.01UI/mL and FVIII inhibitor titer 1320BU/mL. Serological markers, autoimmune and neoplasm study were negative. The patient began treatment with prednisolone 1mg/kg/day and activated prothrombin complex concentrate (aPCC): D1-D5 4000UI 8/8h; D6-D8 5000UI 12/12h; D9-D11 4000UI 12/12h; D12-D14 5000UI 12/12h. At D3 cyclophosphamide 1mg/Kg/day (adjusted GFR) was added. The hematomas showed clinical and radiological improvement with no bleeding signs. At D15, aPCC was stopped due to hemoglobin stability. At D22 laboratory assessment showed a slow decrease of aPTT(86,4s), FVIII inhibitor titer(973BU/mL). On D31, patient complained of shoulder pain and it was noted a new huge hematoma. Laboratory testing showed hemoglobin 8.8g/dL, increased inflammatory parameters and SARS-CoV-2 positive PCR. CT-scan identified a collection with fluid/gas(20x59x99mm) in right interpectoral region extended to gleno-umeral joint (septic arthritis). Broad spectrum antibiotic therapy was started, but on D37, patient died due to ESBL Klebsiella-urosepsis.

Conclusion(s): AHA etiology is only identified in 50% of cases. The outcome of this case report, not related with bleeding, rises the issue of choose an aggressive immunosupression in elderly patients with comorbidities with controlled bleeding, due to related serious infections.