Mieloma múltiple en Chile. Uso de drogas noveles en dos centros nacionales

Background: The incorporation of novel drugs, such as proteasome inhibitors and immunomodulators, improved considerably the survival of patients with multiple myeloma. Aim: To evaluate the effect on survival of proteasome inhibitors and immunomodulators in patients with multiple myeloma in two national hospitals. Material and Methods: Review of clinical records from two hospitals of Santiago. Epidemiological, clinical, laboratory and therapeutic data was obtained from 144 patients with multiple myeloma diagnosed between 2002 and 2016. Results: Information was retrieved from 78 patients at one center and from 66 at the other center. The mean age at diagnosis was 58 and 62 years, the proportion of males was 53% and 52%, and presentation at stage III was 34% and 46%, respectively. The use of novel drugs, mainly bortezomib, was 90% in one of the centers and 3% in the other one. The use of autologous stem-cell transplantation was 47% and 3% respectively. The median overall survival of patients from the centers with and without access to novel drugs was 117 and 71 months respectively (p < 0.05). The five-year overall survival was 93 and 43% respectively (p < 0.05). Conclusions: The use of novel drugs, especially bortezomib, and autologous stem-cell transplantation significantly improved the survival of multiple myeloma patients treated in national hospitals. It is necessary to include them as a first line treatment.

Infección por SARS-CoV-2. Una nueva enfermedad endotelial trombo-inflamatoria

Coronavirus disease 19 is characterized by an intense inflammatory response and a high incidence of thrombotic events. Autopsy studies show severe endothelial injury associated with thrombosis of the alveolar capillaries of the lungs and other organs. In the pathophysiology of this disease endothelial injury and dysfunction, inflammation and thrombosis are key factors for the development of severe phenotypes. Therefore, we should consider this entity as a systemic endothelial disease in which an obstructive microvascular syndrome secondary to an intense thrombo-inflammatory response leads to acute respiratory insufficiency and multiorgan failure. Heparin is an excellent drug to treat Covid-19 patients due to its anticoagulant, anti-inflammatory, antiviral and endothelial effects. Clinical guidelines agree that the use of heparin thromboprophylaxis is a component of Covid-19 coagulopathy treatment, even though the dose and duration of treatments are not well defined.

Trombocitopenia hereditaria relacionada a gen MYH-9: primera familia reportada en Chile con diagnóstico molecular: caso clínico / Hereditary thrombocytopenia associated with a mutation in the MYH-9 gene: report of one case

We report a 51-year-old female who had a first episode of thrombocytopenia at 23 years of age during a pregnancy. At the age of fifty, a hysterectomy was indicated due to a metrorrhagia: a platelet count of 21,000/ul was detected. She was treated with eltrombopag with a good response. The family history of the patient revealed the presence of thrombocytopenia in several family members. Suspecting a hereditary thrombocytopenia, a genetic study revealed a mutation in the MYH-9 gene. This mutation can be suspected when there is a family history of thrombocytopenia with autosomal dominant inheritance, macrothrombocytopenia and in this particular case, due to the response to thrombopoietin receptor agonist, eltrombopag.

Hematoma subcapsular esplénico en paciente portador de rasgo falciforme / Sub capsular splenic hematoma in a sickle cell trait carrier. Case report

Drepanocytic anemia is an uncommon hereditary disease in Chile. The heterozygous state of drepanocytic anemia or "sickle trait" has a frequency of 8 percent among Afro-Americans. A small number of patients carrying hemoglobin S are homozygous, with clinical manifestations of hemolytic anemia and thrombotic disease. Sickle trait is usually asymptomatic. We report a 59-year-old male who presented an acute abdominal pain and dyspnea while staying at high altitude. Six days later, an angio CAT scan showed the presence of a subcapsular splenic hematoma that was managed conservatively. Sickle cell induction with sodium metabisulphite was positive. Hemoglobin electrophoresis confirmed the sickle trait.

La resistencia a aspirina en pacientes cardiovasculares chilenos es frecuente / Aspirin resistance is frecuent in chilean cardiovascular patients

Introducción: La aspirina se usa frecuentemente para la prevención y tratamiento de eventos isquémicos cardiovasculares. Estudios en población anglosajona han mostrado que entre 5-9 por ciento de los pacientes presentan resistencia total a la acción antiplaquetaria de aspirina. Objetivos: Conocer la prevalencia de resistencia a aspirina en enfermos cardiovasculares chilenos. Evaluar factores asociados a la resistencia. Pacientes y Métodos: Se estudian 68 pacientes (21 mujeres, ± 10 años) con enfermedades cardiovasculares estables usuarios de aspirina (100-325 mg/día). Se evalúan variables clínicas y de coagulación básica. Se estudió la agregación plaquetaria con agregómetro óptico en plasma rico en plaquetas con 3 agonista. Se definió Resistencia Total a la aspirina como. 1) agregación (20 por ciento con ácido araquidónico y 2) agregación > 70 por ciento con ADP y/o colágeno. Resultados: Siete pacientes (10,3 por ciento, IC 95 por ciento = 4,2-20,1 por ciento) cumplieron ambos criterios y se consideraron como resistentes totales, 34 (50 por ciento, IC 95 por ciento = 37,6-62,4 por ciento) cumplieron con sólo un criterio y se consideraron como resistentes parciales, y los 27 pacientes restantes (39,7 por ciento, IC 95 por ciento = 28,0-52,3 por ciento) no cumplieron con ninguno de los criterios y se clasificaron como respondedores a AAS. Hubo una mayor frecuencia de fumadores (55,6 vs 23,7 por ciento) en los pacientes con Resistencia Total, pero no alcanzó significación estadística (p = 0,06). Conclusiones: La mayoría de los pacientes cardiovasculares presentan algún grado de resistencia al AAS y un 10,3 por ciento presentan Resistencia Total. No encontramos diferencias significativas entre los los pacientes con y sin resistencia total a aspirina.

Mucormicosis en un paciente con leucemia aguda monoblástica / Mucormicosis in a patient with acute monoblastic leucemia

La mucormicosis es una enfermedad inusual que se presenta en pacientes diabéticos quetoacidóticos y/o inmunodeprimidos. Se comunica el segundo caso en Chile de mucormicosis asociado a candidiasis en un paciente con leucemia aguda monoblástica, que se manifestó por fiebre y compromiso ocular, tratándose con Anfotericina B en forma tardía, sin obtenerse mejoría. La importancia de este caso clínico radica en el hecho de que en un paciente inmunodeprimido, cualquiera que sea su causa, ante la presencia de un cuadro inflamatorio paranasal o periocular debe plantearse el diagnóstico de una mucormicosis. No debe esperarse el resultado de un cultivo o de la biopsia para iniciar de inmediato un tratamiento antimicótico