RESUMEN
The International Prognostic Index (IPI) is the most widely used score for non-Hodgkin lymphoma but lacks the ability to identify a high-risk population in diffuse large B-cell lymphoma (DLBCL). Low absolute lymphocyte count and high monocytes have proved to be unfavourable factors. Red-cell distribution width (RDW) has been associated with inflammation and beta-2 microglobulin (B2M) with tumour load. The retrospective study included 992 patients with DLBCL treated with R-CHOP. In the multivariate analysis, age, Eastern Cooperative Oncology Group performance status (ECOG-PS), stage, bulky mass, B2M, RDW, and lymphocyte/monocyte ratio (LMR) were independently related to progression-free survival (PFS). A new prognosis score was generated with these variables including age categorized into three groups (0, 1, 2 points); ECOG ≥ 3-4 with two; stage III/IV, bulky mass, high B2M, LMR < 2·25 and RDW > 0·96 with one each; for a maximum of 9. This score could improve the discrimination of a very high-risk subgroup with five-year PFS and overall survival (OS) of 19% and 24% versus 45% and 59% of R (revised)-IPI respectively. This score also showed greater predictive ability than IPI. A new score is presented including complete blood cell count variables and B2M, which are readily available in real-life practice without additional tests. Compared to R-IPI, it shows a more precise high-risk assessment and risk discrimination for both PFS and OS.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Recuento de Células Sanguíneas/métodos , Linfocitos/metabolismo , Linfoma de Células B Grandes Difuso/sangre , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Monocitos/metabolismo , Microglobulina beta-2/sangre , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Protocolos de Quimioterapia Combinada Antineoplásica/farmacología , Ciclofosfamida/farmacología , Ciclofosfamida/uso terapéutico , Doxorrubicina/farmacología , Doxorrubicina/uso terapéutico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Prednisona/farmacología , Prednisona/uso terapéutico , Pronóstico , Factores de Riesgo , Rituximab/farmacología , Rituximab/uso terapéutico , Vincristina/farmacología , Vincristina/uso terapéutico , Adulto JovenRESUMEN
El síndrome de Kostmann o agranulocitosis congénita infantil es un trastorno inmunológico hereditario caracterizado por una disminución severa del valor absoluto de neutrófilos; así como la aparición de infecciones piogénas recurrentes en los primeros años de vida. Se describe el caso de un lactante menor masculino de 7 meses de edad, quien ingresa al Hospital de Niños "J.M. de Los Ríos" por presentar fiebre, infección pulmonar y neutropenia. El diagnóstico de síndrome de Kostmann o agranulocitosis congénita se basa por los hallazgos en el aspirado y la biopsia de médula; y el tratamiento con factor estimulante de colonias granulocíticas evitó nuevas infecciones bacterianas