[Secondary hemophagocytic syndrome due to recurrent infections in a severely burned patient]. / Síndrome hemofagocítico secundario a infecciones graves en un quemado.

The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.

Selinexor in Patients from Argentina with Multiple Myeloma Treated with Multiple Prior Therapies: A Case Series.

BACKGROUND Numerous treatment options are available for patients with multiple myeloma (MM). Because of the course of the disease, most patients will experience serial relapse or the MM will become refractory to most of these treatments, leaving patients with few or no treatment options over time. Selinexor, a treatment with a novel mechanism of action, is an oral selective inhibitor of nuclear export (SINE) compound that blocks exportin 1, the major nuclear exporter of tumor suppressor proteins. CASE REPORT In this case series, we report on treatment with the weekly oral administration of selinexor combined with bortezomib and dexamethasone (XVd) in 3 patients from Argentina who were heavily treated (5-7 prior therapies) for MM that relapsed or was refractory to each previous treatment. Two patients had the high-risk cytogenetic abnormality del(17p). All 3 patients experienced efficacy with XVd reaching a best response of partial response or very good partial response. These responses were consistent with those of patients from the BOSTON study who were treated with XVd but were less heavily pretreated (1-3 prior therapies) and had a shorter median time since diagnosis of MM (7 years vs 3.7 years). The 3 patients experienced adverse events (AEs) that included nausea, thrombocytopenia, asthenia, and fatigue, which were similar to the most commonly reported AEs associated with selinexor treatment. CONCLUSIONS With its oral administration, novel mechanism of action, and responses in heavily pretreated patients, selinexor may help to address an important clinical need in the treatment of patients with relapsed/refractory MM.

Mild COVID-19 Illness as a Risk Factor for Venous Thromboembolism.

Introduction From the beginning of the current coronavirus disease 2019 (COVID-19) pandemic, there is cumulative evidence suggesting that patients hospitalized due to this disease are at a high risk for venous thromboembolism (VTE). The association between mild non-hospitalized illness and VTE is unclear. The purpose of this research is to assess the association between VTE and mild COVID-19 infection. Methods A case-control study was conducted. The cases were adult patients diagnosed with VTE from March 1, 2020 to March 31, 2021. The controls were randomly chosen adult patients who required healthcare services that were equivalent to those of the cases, for any cause, during the same time period, without a VTE diagnosis. To assess the association between mild COVID and VTE, a multivariate logistic regression analysis was conducted, considering other thromboembolic risk variables, such as age, gender and active cancer, among others. A p-value <0.05 was considered statistically significant. Results A total of 186 cases and 475 controls were analyzed. There were 21 (11.3%) and 31 (6.5%) patients infected with mild COVID-19 in the previous three months in the groups of cases and controls, respectively. Mild COVID-19 infection was statistically significant as a risk factor for VTE both in the univariate analysis and in the multivariate analysis, OR=1.82 (95% CI 1.02-3.26) and OR=2.62 (95% CI 1.34-5.13), respectively. Conclusion Mild COVID-19 infection might be an independent risk factor for VTE. We conclude that the results suggest some thromboprophylaxis strategy should be considered in certain patients with COVID-19 infection in an outpatient fashion.

Síndrome hemofagocítico secundario a infecciones graves en un quemado / Secondary hemophagocytic syndrome due to recurrent infections in a severely burned patient

El síndrome hemofagocítico constituye una entidad infrecuente, heterogénea, subdiagnosticada, y muchas veces fatal. En los casos secundarios, los desencadenantes pueden ser numerosos, tales como infecciones, fármacos, enfermedades autoinmunes y neoplasias. El mecanismo fisiopatogénico se explica por la presencia de una función disminuida o defectuosa de células NK y linfocitos T citotóxicos, que resulta en una activación inmune inefectiva y descontrolada, conduciendo al daño celular, falla multiorgánica y proliferación macrofágica con hemofagocitosis. Existen diferentes opciones terapéuticas, mayormente combinaciones de citostáticos y esteroides, cuyo objetivo es la supresión de la respuesta inmune descontrolada. Ocasionalmente, la condición clínica de algunos pacientes con síndrome hemofagocítico impide la utilización de esquemas terapéuticos intensivos. Comunicamos el caso de un paciente quemado grave, que reúne los criterios diagnósticos de síndrome hemofagocítico, quien presentó una evolución favorable con el tratamiento combinado de esteroides e inmunoglobulinas endovenosas.

The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.