RESUMO
INTRODUCTION: Diagnosis of AL amyloidosis can be complicated by the diversity and the absence of specificity of symptoms. CASE REPORT: We report a patient who presented with a non-traumatic hepatic hematoma, leading to the discovery of hepatic amyloidosis secondary to probable multiple myeloma. The originality of our report lies in the discovery of two acquired abnormalities of haemostasis: a factor X deficiency and an acquired von Willebrand syndrome, by a likely inhibitor. CONCLUSION: Our case report is a reminder of the importance of haemostasis analysis in AL amyloidosis.
Assuntos
Amiloidose/complicações , Hematoma/etiologia , Hepatopatias/etiologia , Amiloidose/diagnóstico , Deficiência do Fator X/complicações , Deficiência do Fator X/diagnóstico , Feminino , Hematoma/diagnóstico , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina , Hepatopatias/diagnóstico , Pessoa de Meia-Idade , Doenças de von Willebrand/complicações , Doenças de von Willebrand/diagnósticoAssuntos
Dor Abdominal/imunologia , Anticorpos Anticardiolipina/imunologia , Síndrome Antifosfolipídica/diagnóstico , Medula Óssea/patologia , Inibidor de Coagulação do Lúpus/imunologia , Complicações na Gravidez/imunologia , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/imunologia , Síndrome Antifosfolipídica/terapia , Biomarcadores/sangue , Medula Óssea/imunologia , Cesárea , Diagnóstico Diferencial , Feminino , Humanos , Necrose , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , Terceiro Trimestre da Gravidez , Trombocitopenia/imunologia , Resultado do TratamentoRESUMO
BACKGROUND AND OBJECTIVES: Inherited factor VII (FVII) deficiency is a rare bleeding disorder characterized by a poor relationship between reported FVII clotting activity (FVII:C) and bleeding tendency. Our study was aimed at defining biological parameters that are possibly predictive for bleeding risk in this condition. DESIGN AND METHODS: Forty-two FVII-deficient patients (FVII:C <30%) were classified into two opposite clinical groups defined as severe and non-or-mild bleeders. For each patient, plasma samples were collected and then investigated for FVII:C (using a sensitive method and human recombinant thromboplastin as the reagent), FVII antigen, activated FVII coagulant activity (FVIIa:C) and the free-form of tissue factor pathway inhibitor. RESULTS: None of these tests could be used as highly accurate predictors of bleeding. Nevertheless, both FVII:C and FVIIa:C differed significantly between the two clinical groups. Using ROC-curve analysis, two critical values of 8% and 3mIU/mL for FVII:C and FVIIa:C, respectively, could be proposed to discriminate between severe bleeders and non-or-mild bleeders. INTERPRETATION AND CONCLUSIONS: A highly accurate diagnostic test for predicting bleeding tendency in inherited FVII deficiency still eludes definition, highlighting the fact that factors other than FVII itself interfere with the expression of bleeding phenotypes in this condition. Nevertheless, potential critical values using sensitive FVII:C and FVIIa:C methods may be useful in clinical laboratories for FVII-deficient patients. Those patients with FVII:C levels higher than 8% FVII:C or FVIIa:C higher than 3 mIU/mL, with no other hemostatic defect, seem to have a minimal risk of severe bleeding. Extended clinical studies are needed to support these findings.