RESUMO
Factor V inhibitors are a rare cause of life-threatening bleeding. We present a case of an acquired factor V inhibitor likely caused by coronavirus disease 2019 infection. Bleeding was manifested by severe anemia requiring frequent red-cell transfusion, left psoas muscle hematoma, and left retroperitoneal cavity hematoma. Factor V activity was less than 1% and the factor V inhibitor titer was 31.6 Bethesda units. Severe acute respiratory syndrome coronavirus 2 RNA testing of the nasopharynx was positive 2 weeks before presentation and continued to be positive for 30 days. The patient failed treatment with intravenous immunoglobulin and dexamethasone. Three cycles of plasmapheresis with fresh frozen plasma replacement resulted in correction of the bleeding and laboratory coagulopathy. This is the first reported case of a factor V inhibitor in a coronavirus disease 2019 patient and suggests that plasmapheresis may be a successful treatment strategy.
Assuntos
Autoanticorpos/biossíntese , COVID-19/sangue , Fator V/imunologia , Transtornos Hemorrágicos/etiologia , SARS-CoV-2 , Idoso de 80 Anos ou mais , Anemia/etiologia , Anemia/terapia , Anticorpos Antivirais/sangue , Especificidade de Anticorpos , Autoanticorpos/imunologia , COVID-19/complicações , COVID-19/diagnóstico , COVID-19/imunologia , Terapia Combinada , Comorbidade , Diagnóstico Tardio , Dexametasona/uso terapêutico , Transfusão de Eritrócitos , Fator V/antagonistas & inibidores , Feminino , Hematoma/etiologia , Transtornos Hemorrágicos/tratamento farmacológico , Transtornos Hemorrágicos/terapia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Inibidor de Coagulação do Lúpus/sangue , Octreotida/uso terapêutico , Plasma , Plasmaferese , SARS-CoV-2/imunologia , Vitamina K/uso terapêuticoRESUMO
: Combined factor V and factor VIII deficiency is an extremely rare bleeding disorder for which research is lacking. We present the case of a 33-year-old man requiring repeat mitral valve replacement. A multidisciplinary team approach was utilized to minimize his risk of bleeding which included the use of plasma exchange, intravenous factor replacement, and platelet transfusion. This approach created an operative experience that did not require blood transfusion or the use of other hemostatic medications.
Assuntos
Deficiência do Fator V/tratamento farmacológico , Hemofilia A/tratamento farmacológico , Cirurgia Torácica/métodos , Adulto , Testes de Coagulação Sanguínea , Humanos , MasculinoRESUMO
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder associated with hematoproliferative disorders, autoimmune conditions, neoplasia and cardiovascular disorders that often present a diagnostic challenge. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of AVWS that typically presents later in life with mucocutaneous or postsurgical bleeding and multimers consistent with type I or II von Willebrand disease (VWD). Here, we present the case of a patient with a 32-year history of type III VWD that was ultimately found to be AVWS related to an IgG MGUS. In this case report, we highlight the diagnostic challenges of AVWS to ensure proper identification and potentially lifesaving treatment of this rare disorder.