RESUMO
We present a rare case of a man in his 40s who presented with bilateral lower extremity necrosis. After an extensive workup, he was diagnosed with type I cryoglobulinaemia (TIC) based on severe vaso-occlusive symptoms, presence of serum cryoglobins and tissue biopsy showing small-vessel vasculitis. Treatment was multimodal and targeted both his underlying lymphoproliferative disorder (monoclonal gammopathy of undetermined significance) and inflammatory state. Steroids, plasmapheresis and immunotherapy were administered with temporary remission of symptoms. After discharge, patient continued to repeatedly present with progressive bilateral lower extremity necrosis and new upper extremity digital necrosis necessitating further pharmacological treatment and surgical intervention-bilateral above the knee amputation and multiple digital hand amputations. This case illustrates a severe example of TIC where diagnosis was difficult due to atypical presentation, and disease was refractory to multimodal therapies necessitating surgical intervention to achieve temporary remission.
Assuntos
Crioglobulinemia , Masculino , Humanos , Crioglobulinemia/complicações , Crioglobulinemia/diagnóstico , Crioglobulinemia/terapia , Amputação Cirúrgica , Biópsia , Terapia Combinada , NecroseRESUMO
BACKGROUND: Myocardial recovery following left ventricular assist device (LVAD) implantation has been of interest in transplant candidates with non-ischemic cardiomyopathy but is rare. Evidence suggests that a combination of left ventricular unloading and pharmacologic reverse remodeling is beneficial. Recovery in non-transplant candidates (i.e., destination therapy [DT]) patients is believed to be even rarer. METHODS: All DT LVADs between January 1, 2017 and November 23, 2020 were reviewed. All patients were subjected to an institutional protocol consisting of combined pharmacologic remodeling and mechanical unloading with proactive screening for recovery. The primary outcome of interest was the cumulative incidence of myocardial recovery. Baseline characteristics and operative outcomes were compared between recovered and non-recovered DT patients using non-parametric tests to identify predictive factors. RESULTS: A total of 49 patients received DT LVADs. Nine patients were identified as myocardial recovery candidates using the protocol screening criteria. Overall, 11 patients underwent formal confirmatory testing for recovery, of which 10 were deemed recovered and underwent LVAD explant, defunctionalization, or transplantation. 37.5% of patients that had a concomitant coronary artery bypass during LVAD implantation achieved recovery. An equal proportion of ischemic and non-ischemic cardiomyopathy patients achieved recovery. The cumulative incidence of myocardial recovery was 25.1% at 36 months. No factors were identified as being predictive of recovery. CONCLUSION: Myocardial recovery in DT LVAD patients can be achieved at a higher rate than previously reported. Revascularization at the time of LVAD is safe and may be beneficial. LVAD therapy may not be the final destination in these patients.