RESUMEN
A small subset of patients with antiphospholipid syndrome (APS) may develop widespread thrombotic disease with organ damage, referred to as catastrophic APS (CAPS) that is associated with a high mortality. Medical therapy typically involves a combination of anticoagulation, systemic glucocorticoids, plasmapheresis, and intravenous immune globulin (IVIG). There is currently no consensus for the management of refractory cases of CAPS. However, monoclonal antibodies such as rituximab and eculizumab have shown some benefits. Herein, we present a 29-year-old female with previous pulmonary embolism who presented with necrotic left toes and was eventually diagnosed with refractory CAPS, successfully treated with Plasmapheresis and Rituximab. With this case report, we hope to encourage the usage of Rituximab in the management of CAPS.
RESUMEN
BACKGROUND: Primary cardiac sarcomas (PCS) are extremely rare malignant tumors involving the heart. Only isolated case reports have been described in the literature over different periods of time. This pathology has been associated with a dismal prognosis and given its rarity; treatment options are very limited. Furthermore, there are contrasting data about the effectiveness of current treatment modalities in improving the survival of patients with PCS, including surgical resection which is the mainstay of therapy. There is a paucity of data on the epidemiological characteristics of PCS. This study has the objective of investigating the epidemiologic characteristics, survival outcomes, and independent prognostic factors of PCS. METHODS: A total of 362 patients were ultimately registered in our study from the Surveillance, Epidemiology, and End Results (SEER) database. The study period was from 2000 to 2017. Demographics such as clinical characteristics, overall mortality (OM), and PCS-specific mortality (CSM) were taken into account. A p value of <0.1 in the univariate analysis leads to the incorporation of the variable into multivariate analysis adjusting for covariates. Adverse prognostic factors were represented by a Hazard Ratio (HR) greater than one. The five-year survival analysis was carried out using the Kaplan-Meier method and the log-rank test was used to compare survival curves. RESULTS: Crude analysis revealed a high OM in age 80+ (HR = 5.958, 95% CI 3.357-10.575, p < 0.001), followed by age 60-79 (HR = 1.429, 95% CI 1.028-1.986, p = 0.033); and PCS with distant metastases (HR = 1.888, 95% CI 1.389-2.566, p < 0.001). Patients that underwent surgical resection of the primary tumor and patients with malignant fibrous histiocytomas (HR = 0.657, 95% CI 0.455-0.95, p = 0.025) had a better OM (HR = 0.606, 95% CI 0.465-0.791, p < 0.001). The highest cancer-specific mortality was observed in age 80+ (HR = 5.037, 95% CI 2.606-9.736, p < 0.001) and patients with distant metastases (HR = 1.953, 95% CI 1.396-2.733, p < 0.001). Patients with malignant fibrous histiocytomas (HR = 0.572, 95% CI 0.378-0.865, p = 0.008) and those who underwent surgery (HR = 0.581, 95% CI 0.436-0.774, p < 0.001) had a lower CSM. Patients in the age range 80+ (HR = 13.261, 95% CI 5.839-30.119, p < 0.001) and advanced disease with distant metastases (HR = 2.013, 95% CI 1.355-2.99, p = 0.001) were found to have a higher OM in the multivariate analyses adjusting for covariates). Lower OM was found in patients with rhabdomyosarcoma (HR = 0.364, 95% CI 0.154-0.86, p = 0.021) and widowed patients (HR = 0.506, 95% CI 0.263-0.977, p = 0.042). Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups, and lower mortality in patients with Rhabdomyosarcoma. CONCLUSION: In this United States population-based retrospective cohort study using the SEER database, we found that cardiac rhabdomyosarcoma was associated with the lowest CSM and OM. Furthermore, as expected, age and advanced disease at diagnosis were independent factors predicting poor prognosis. Surgical resection of the primary tumor showed lower CSM and OM in the crude analysis but when adjusted for covariates in the multivariate analysis, it did not significantly impact the overall mortality or the cancer-specific mortality. These findings allow for treating clinicians to recognize patients that should be referred to palliative/hospice care at the time of diagnosis and avoid any surgical interventions as they did not show any differences in mortality. Surgical resection, adjuvant chemotherapy, and/or radiation in patients with poor prognoses should be reserved as palliative measures rather than an attempt to cure the disease.
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Background In this study, we aimed to provide a descriptive overview of the utilization of hematopoietic stem cell transplantation (HSCT) for the treatment of acute myeloid leukemia (AML), determine the rates of HSCT use stratified by patients' demographic characteristics, and measure the hospitalization outcomes. Methodology We conducted a cross-sectional study using the Nationwide Inpatient Sample (NIS) obtained from hospitals in the United States. Our sample included 21,385 adult patients (aged ≥18 years) with a primary discharge diagnosis of AML. The sample was further grouped by inpatients who were managed with HSCT and chemotherapy as the primary procedure. We compared the demographic characteristics and hospital outcomes in AML inpatients across treatment cohorts by performing descriptive statistics and Pearson's chi-square test. Next, we measured the differences in continuous variables (length of stay and cost) using the analysis of variance (ANOVA). All analyses were conducted using SPSS version 26 (IBM Corp., Armonk, NY, USA). Results The hospital-based utilization rate of HSCT was 0.4% in AML inpatients. The utilization rate of HSCT was higher in females (0.5%), African Americans (0.6%), those with median household incomes above the 50th percentile (0.5%), and those covered by private insurance (0.8%). A significantly higher proportion of AML inpatients with HSCT had depression (22.2% vs. 11.4% in total). AML inpatients receiving HSCT had significantly longer hospitalization stays and higher treatment costs than those receiving chemotherapy. The all-cause inpatient mortality was 11.6% in AML inpatients. Statistically, there were no significant differences by treatment. Conclusions HSCT appears to be underutilized for the treatment of AML. This treatment had a higher utilization rate in females and those from high-income families and was covered by private insurance. The utilization of chemotherapy and HSCT did not significantly differ in the presence of comorbidities, except for depression and hypertension having a higher utilization of HSCT.