RÉSUMÉ
We report a case of progressive light-chain amyloidosis (otherwise known as AL amyloidosis) with acquired factor X (aFX) deficiency with a complete haematological response and rapid normalisation of FX levels following daratumumab monotherapy. To our knowledge, this is the first case report documenting successful treatment with daratumumab of aFX deficiency secondary to AL amyloidosis. The patient responded well to this therapy, with excellent symptomatic and quality of life improvements as well as a reduction in bleeding manifestations. This case highlights the value in considering daratumumab treatment when AL amyloidosis is complicated by FX deficiency.
Sujet(s)
Amyloïdose , Déficit en facteur X , Amylose à chaine légère d'immunoglobuline , Amyloïdose/traitement médicamenteux , Déficit en facteur X/complications , Déficit en facteur X/traitement médicamenteux , Hémorragie , Humains , Amylose à chaine légère d'immunoglobuline/traitement médicamenteux , Qualité de vieRÉSUMÉ
We report a case of chronic Q fever presenting with catastrophic bleeding from an infected abdominal aortic aneurysm causing a primary aortoduodenal fistula in an 80-year-old retired farmer. This presentation is rarely reported in literature and only through case reports. Early diagnosis and definitive surgery were critical to a successful outcome. Serological diagnosis of Q fever was initiated on the patient's past exposure to animal reservoirs. Complicating the case was ongoing gastrointestinal bleeding postsurgery, with multiple endoscopies undertaken before a culprit remnant fistula was found. This case highlights the value in considering Coxiella burnetii as an underlying cause in patients with known risk factors presenting with primary aortoduodenal fistulas. Though rare, it represents a readily treatable cause.