RESUMO
In localized light chain amyloidosis (locAL), amyloidogenic light chains (aLC) are produced and deposited locally by a B-cell clone. We present 293 patients with immunohistochemically confirmed locAL. Lung (nodular pulmonary) with 63 patients was the most involved organ. The aLC was λ in 217 cases (κ:λ ratio 1:3). A local B-cell clone was identified in 30% of cases. Sixty-one (21%) had a concomitant autoimmune disorder (cAD). A monoclonal component (MC) were present in 101 (34%) patients and were more frequent in subjects with cAD (51% vs 34%; P = .03). Cigarette smoking was more prevalent in lung locAL (54% vs 37%; P = .018). After a median follow-up of 44 months, 16 patients died and 5- and 10-years locAL progression-free survival (PFS) were 62% and 44%. Interestingly, locAL-PFS was shorter among patients with an identified clonal infiltrate at amyloid deposition site (40 vs 109 months; P = .02) and multinuclear giant cells and/or an inflammatory infiltrate resulted in longer locAL-PFS in lung involvement (65 vs 42 months; P = .01). However, no differences in locAL PFS were observed in patients with cAD, a MC and involved organ site. Treatment was administered in 163 (54%) patients and was surgical in 135 (46%). Median locAL-PFS after first treatment was 56 months. Responders had longer locAL-PFS (78 vs 17 months; P < .001). Three patients with lung locAL and a MC were diagnosed as systemic AL amyloidosis at follow-up. In summary, locAL pathogenesis seems to be heterogeneous and the clonal infiltrate leads local progression.
RESUMO
BACKGROUND: Burns that involve the perineum, buttocks and genitals (PBG) have been associated with more challenging therapeutic needs and worse clinical outcomes. We aimed to investigate whether PBG burns are an independent predictor for mortality, morbidity and complications in a large, heterogenous patient collective and in comparison to patients without PBG burns. PATIENTS AND METHODS: Patients admitted to a level one burn center between August 2014 and July 2022 were included and stratified based on the presence of PBG burns on admission (PBG & control group = CTR). Demographic baseline data, burn aetiology, inhalation trauma (IHT), full-thickness burns (FT), number of operations (NOR), mortality, length of ICU stay (LOS-ICU), length of in-hospital stay (LOHS) and bacteraemia were assessed to compare key clinical characteristics and outcomes between the groups. Multivariate regression analyses and a 1:1 propensity score matching were conducted for key clinical outcomes. RESULTS: A total of 1024 patients were included in the analysis (PBG: n = 227; CTR: n = 797). PBG burns were older (median (IQR) 54 (34-72) vs. 44, (30-61) years, p < 0.0001), more frequently female (35% vs. 23%, p = 0.002) presented with larger total body surface area (TBSA) burns overall (27 (32-39) vs. 10 (13-15) %, p < 0.0001) and sustained FT burns more frequently (69% vs. 26% p < 0.0001). Scald burns were more frequently the cause of PBG burns (45% vs. 15%, p < 0.0001), PBG patients needed twice as many surgical procedures (Mean (SD) 2 (2.84) vs. 1 (1.6), p < 0.0001) as CTR. In multivariate analyses, a significant correlation was identified between length of ICU stay and presence of PBG burns. Following strict cohort matching to account for sex, age, cause of burn, TBSA %, presence of FT burn, inhalation trauma and bacteraemia, PBG burns were an independent predictor for mortality (p = 0.0003). CONCLUSION: PBG burns are at risk for prolonged intensive care, hospitalization and complications during treatment. Furthermore, the presence of PBG burns appears to be a risk factor for mortality, irrespective of patient age, TBSA affected and other relevant covariates.