RESUMEN
The purpose of this study was to evaluate the frequency of osteoporosis (OP) in patients with Gaucher disease (GD) in Argentina. GD patients from 28 centers were consecutively included from April 2012 to 2014. Bone mineral density (BMD) was determined by dual X-ray absorptiometry in the lumbar spine and the femoral neck or the total proximal femur for patients ≥20 yr of age, and by whole-body scan in the lumbar spine in patients <20 yr of age. In children, mineral density was calculated using the chronological age and Z height. OP diagnosis was determined following adult and pediatric official position of the International Society for Clinical Densitometry. A total of 116 patients were included, of which 62 (53.5%) were women. The median age was 25.8 yr. All patients received enzyme replacement therapy, with a median time of 9.4 yr. Normal BMD was found in 89 patients (76.7%), whereas low bone mass (LBM) or osteopenia was found in 15 patients (13%) and OP in 12 patients (10.3%). The analysis of the pediatric population revealed that 4 patients (9.3%) had LBM and 3 (7%) had OP (Z-score ≤ -2 + fractures height-adjusted by Z), whereas in the adult population (n = 73), 11 patients (15%) had LBM or osteopenia and 9 (12.3%) had OP. Bone marrow infiltration and the presence of fractures were significantly correlated with the presence of OP (p = 0.04 and <0.001, respectively). This is the first study in Argentina and in the region describing the frequency of OP or LBM in GD patients treated with imiglucerase using the official position of the International Society for Clinical Densitometry.
Asunto(s)
Absorciometría de Fotón , Enfermedad de Gaucher/complicaciones , Osteoporosis/complicaciones , Osteoporosis/diagnóstico por imagen , Absorciometría de Fotón/métodos , Adolescente , Adulto , Anciano , Argentina/epidemiología , Densidad Ósea , Enfermedades Óseas Metabólicas/complicaciones , Enfermedades Óseas Metabólicas/diagnóstico por imagen , Enfermedades Óseas Metabólicas/epidemiología , Niño , Femenino , Cuello Femoral/diagnóstico por imagen , Enfermedad de Gaucher/epidemiología , Humanos , Vértebras Lumbares/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Osteoporosis/epidemiología , Adulto JovenRESUMEN
A new solitary extramedullary plasmacytoma of nasal localization meeting the conditions for definition is reported. The patient was treated with surgery and irradiation, which controlled the disease and produced excellent cosmetic results. The bibliography is reviewed and the pathologic, clinical, and evolutive characteristics are discussed.
Asunto(s)
Neoplasias Nasales/diagnóstico , Plasmacitoma/diagnóstico , Anticuerpos Monoclonales/inmunología , Terapia Combinada , Humanos , Masculino , Persona de Mediana Edad , Nariz/diagnóstico por imagen , Nariz/patología , Nariz/cirugía , Neoplasias Nasales/inmunología , Neoplasias Nasales/terapia , Plasmacitoma/inmunología , Plasmacitoma/terapia , Tomografía Computarizada por Rayos XRESUMEN
In this study, 54 patients with relapsed or refractory non-Hodgkin's lymphoma (NHL) were treated in a phase II, multicentric trial with ifosfamide-mesna 1500 mg/m2 IV days 1-3, idarubicin 12 mg/m2 IV day 1 and etoposide 100 mg/m2 IV day 1-3 (MIZE). Overall response was 72%; complete response (CR) and partial response (PR) were 46% and 26% respectively. In Stage I-II pts CR was 59% and in Stage III-IV pts CR was 40.5%. Patients who relapsed from an initial CR had a 64% CR rate when treated with MIZE, in contrast to refractory disease's patients who only had 19% CR (p = 0.004). The group of pts that had an objective response (CR + PR) to front line therapy had a 2 year survival rate of 55% compared with none for refractory disease (p = 0.029) after salvage therapy. Median survival for the entire group was 17.5 months. Better survival was seen in pts who were asymptomatic with low levels of LDH, previous CR, non high-grade histology, and limited disease stage at relapse. Toxicity was mainly hematologic: 91.5% had neutropenia, (56.5% grade III-IV), and 9.5% died from infectious complications. Other clinical toxicities including cardiac toxicity were negligible. MIZE chemotherapy was effective in patients with relapsed and refractory lymphoma and showed limited clinical and cardiac toxicity. Myelosupression was the most frequent single toxicity.