The Latin-American Experience in POEMS Syndrome: A Study of the GELAMM (Grupo de Estudio Latinoamericano de Mieloma Múltiple).

INTRODUCTION: POEMS syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The acronym refers to the following features: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal paraproteinemia, and skin changes. METHODS: The study was conducted at 24 hematological centers across 8 Latin-American countries. The study included a total of 46 patients {median age was 52 years (interquartile range [IQR]: 42-61.5), 30 males and 16 females} fulfilling the POEMS syndrome criteria diagnosed over a period of 12 years (January 1, 2011, through July 31, 2023). Epidemiological and clinical data were collected in an ad hoc database sent to the members of GELAMM, as well as the Kolmogorov-Smirnov test and Kaplan-Meier estimates. RESULTS: All patients had polyneuropathy and monoclonal gammopathy; 89% had bone marrow plasma cell infiltration, 33% had sclerotic bone lesions. Only 10 patients underwent vascular endothelial growth factor (VEGF) testing in plasma samples. The paraproteinemia was IgG λ in 32% and IgA λ in 30%. 59% patients presented with cutaneous changes, mainly hyperpigmentation, 54% had organomegaly, and 74% endocrinopathy. The median interval from symptom onset to diagnosis was 7.7 months (IQR: 4.0-12.6). 69% of patients received a single line of treatment. The median follow-up period was 25 months (IQR: 9.37-52.0) and the 2-year overall survival rate was 100%. All patients who underwent transplantation (43%) are alive, with a median follow-up of 45.62 months (IQR: 15.46-70). CONCLUSION: This study investigates POEMS syndrome in Latin America and presents an initial overview of the disease in the region. VEGF usage is recommended for accurate diagnosis, but only 7 hematology centers in the region used it. Survival rate in Latin America is comparable with those observed internationally.

Two Hour Evaluation and Referral Model for Shorter Turnaround Times in the emergency department.

OBJECTIVE: The objective of this study was to assess the implementation of a novel ED model of care, which combines clinical streaming, team-based assessment and early senior consultation to reduce length of stay. METHODS: A pre-post-intervention study was used to compare ED performance following an extensive clinical redesign programme. Clinical teams and work sequences were reconfigured to promote the role of the staff specialist, with a focus on earlier decisions regarding disposition. Primary outcome measures were ED length of stay and National Emergency Access Target (NEAT) compliance. Secondary outcomes included referral and workup times, wait times by triage category, ambulance offload times, ward discharges and unit transfers within 24 h of admission, representation within 48 h, and Medical Emergency Response Team (MERT) calls within 24 h of admission. RESULTS: Two seasonally matched 26 week intervals were compared with adjustment for demographics, triage category and arrival by ambulance. Overall, there was an 18.4% rise in NEAT performance (95% confidence interval (CI): 17.7-19.1) while ED length of stay decreased by a total of 86.8 min (95% CI: 83.6-90.1). Time series analysis did not suggest any preexisting trends to explain these results. The average time to referral decreased by 74.7 min (95% CI: 69.8-79.6) and waiting times decreased across all triage categories. Rates of MERT activation and unplanned representation were unchanged. CONCLUSION: A facilitated team leader role for senior doctors can help to reduce length of stay by via early disposition, without significant risks to the patient.

[IgG4-related disease, the new "great mimicker": report of one case]. / Enfermedad relacionada a IgG4, el nuevo "gran simulador": caso clínico.

Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.

Enfermedad relacionada a IgG4, el nuevo "gran simulador": caso clínico / IgG4-related disease, the new "great mimicker": Report of one case

Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.