[Bortezomib in relapsed or refractory multiple myeloma: results in a cohort of 39 patients]. / Bortezomib en mieloma múltiple en recidiva o refractario: resultados en una cohorte de 39 pacientes.

INTRODUCTION: Bortezomib has presently become a significant rescue treatment in multiple myeloma (MM) due to its observed effectiveness and safety in multicenter trials. We have aimed to verify both aspects in a setting of non-selected patients. PATIENTS AND METHODS: This is an observational, prospective study of the cohort of relapsed or refractory MM patients treated with bortezomib in our Department. The variables analyzed were response, its duration, time to the treatment failure (TTF), overall survival (OS), response related conditions and toxicity. Statistical methods used were Fisher's exact test, log rank-test and Kaplan-Meier survival tables. RESULTS: A total of 39 patients, 25 relapsed and 14 refractory to chemotherapy, started the treatment. The mean number of previous treatment was 2.3 and they received an average of 5.8 cycles of bortezomib. Complete response was achieved in 14 patients (36%), partial response in 12 (31%) and minor or no response in 13 ones (33%). Median duration of response was 8 months, median TTF was 10 months and median OS, from the onset of bortezomib was 16.5 months, with a median observation of live patients of 12.5 months. The response was more frequent in males (p = 0.019) and in patients with one previous treatment (p = 0.15). There were no significant differences regarding to TTF when we considered the cause of treatment (relapse or no response to chemotherapy) nor in the number of previous treatment regimes. The most frequent adverse events were reversible thrombocytopenia (31%), polyneuropathy (28%) and asthenia-anorexia (23%). CONCLUSIONS: In our cohort of non-selected, relapsed or refractory MM patients, the observations found in the multicenter randomized trials results regarding response rate and duration, TTF OS and safety of bortezomib therapy were verified.

Bortezomib en mieloma múltiple en recidiva o refractario: resultados en una cohorte de 39 pacientes / Bortezomib in relapsed or refractory multiple myeloma: results in a cohort of 39 patients

Introducción. Bortezomib se ha incorporado como tratamiento de rescate en el mieloma múltiple (MM) por la eficacia y perfil de seguridad observados en estudios multicéntricos. Pretendemos verificar ambos aspectos en un entorno de pacientes no seleccionados. Pacientes y métodos. Estudio observacional prospectivo de los pacientes con MM en recaída o refractarios tratados con bortezomib en nuestro Servicio. Variables analizadas: respuesta, duración, tiempo hasta el fallo del tratamiento (TFT), supervivencia global (SVG) y variables relacionadas con la respuesta y toxicidad. Métodos estadísticos: prueba exacta de Fisher, log rank-test y tablas de supervivencia de Kaplan-Meier. Resultados. Iniciaron el tratamiento 39 pacientes, refractarios a quimioterapia (14) o en recidiva (25), con una media de 2,3 líneas de tratamiento previas, administrándose un promedio de 5,8 ciclos de bortezomib. Alcanzan respuesta completa (RC) 14 pacientes (36%), parcial (RP) 12 (31%), y menor o nula 13 (33%). La duración mediana de la respuesta fue de 8 meses, de TFT 10 meses, y de SVG desde el inicio de bortezomib 16,5 meses, con una mediana de observación de los pacientes vivos de 12,6 meses. La respuesta fue más frecuente en varones (p = 0,019) y en pacientes con sólo una pauta previa de tratamiento (p = 0,15), sin variaciones significativas en TFT al considerar motivo de su instauración (recidiva o refractariedad) o número de pautas previas. Efectos adversos más frecuentes: trombocitopenia reversible (31%), polineuropatía (28%) y astenia-anorexia (23%). Conclusiones. En nuestra serie de pacientes no seleccionados con MM recurrente o refractario se ratifican las observaciones de estudios multicéntricos con bortezomib en la frecuencia, duración de la respuesta, TFT, SVG y perfil de efectos secundarios

Introduction. Bortezomib has presently become a significant rescue treatment in multiple myeloma (MM) due to its observed effectiveness and safety in multicenter trials. We have aimed to verify both aspects in a setting of non-selected patients. Patients and methods: This is an observational, prospective study of the cohort of relapsed or refractory MM patients treated with bortezomib in our Department. The variables analyzed were response, its duration, time to the treatment failure (TTF), overall survival (OS), response related conditions and toxicity. Statistical methods used were Fisher's exact test, log rank-test and Kaplan-Meier survival tables. Results. A total of 39 patients, 25 relapsed and 14 refractory to chemotherapy, started the treatment. The mean number of previous treatment was 2.3 and they received an average of 5.8 cycles of bortezomib. Complete response was achieved in 14 patients (36%), partial response in 12 (31%) and minor or no response in 13 ones (33%). Median duration of response was 8 months, median TTF was 10 months and median OS, from the onset of bortezomib was 16.5 months, with a median observation of live patients of 12.5 months. The response was more frequent in males (p = 0.019) and in patients with one previous treatment (p = 0.15).There were no significant differences regarding to TTF when we considered the cause of treatment (relapse or no response to chemotherapy) nor in the number of previous treatment regimes. The most frequent adverse events were reversible thrombocytopenia (31%), polyneuropathy (28%) and asthenia-anorexia (23%). Conclusions. In our cohort of non-selected, relapsed or refractory MM patients, the observations found in the multicenter randomized trials results regarding response rate and duration, TTF OS and safety of bortezomib therapy were verified

Fiebre mediterránea familiar

La fiebre mediterránea familiar es una enfermedad hereditaria que se caracteriza por la aparición de crisis repetidas y autolimitadas de fiebre y serositis. Indice en algunos grupos étnicos originarios del litoral mediterráneo y su patogenia es mal conocida. Su complicación más temible es la aparición de amiloidosis, que afecta principalmente al riñón, y es causa de síndrome nefrótico y de insuficiencia renal. Puede simular muchas otras enfermedades y no existe ninguna prueba específica para su diagnóstico, que se basa en criterios clínicos y, en ocasiones, en la respuesta a la administración de colchicina. Este fármaco previene las crisis agudas y la aparición o la progresión de la amiloidosis (AU)

[Acute adrenal insufficiency caused by bilateral adrenal hemorrhage as first manifestation of antiphospholipid syndrome. Report of a case and review]. / Insuficiencia suprarrenal aguda por hemorragia suprarrenal bilateral como primera manifestación de un síndrome antifosfolípido. A propósito de un caso y revisión.

Although adrenal hemorrhage is an unusual feature of antiphospholipid syndrome (APS), it can be its first clinical manifestation. The pathogenetic substratum is the thrombosis of the adrenal veins leading to hemorrhagic infarction. Recurrences are frequent in patients with APS-related thrombosis, thus long-term anticoagulation is advocated by most authors.

Insuficiencia suprarrenal aguda por hemorragia suprarrenal bilateral como primera manifestación de un síndrome antifosfolípido. A propósito de un caso y revisión / Acute adrenal insuficiency caused by adrenal hemorrhage as a first manifestation of the antiphospholipid syndrome. A case report and review

La hemorragia suprarrenal (HS) asociada a síndrome antifosfolípido (SAF) en relación con lupus eritematoso sistémico (LES), es poco frecuente. Puede ser la manifestación inicial de la enfermedad. Se invoca como patogenia la trombosis en las venas de drenaje suprarrenales e infarto hemorrágico secundario. El riesgo de trombosis recurrente en estos pacientes es alto. Se aconseja por ello la anticoagulación indefinida (AU)

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[Population reference ranges and determinants of plasma homocysteine levels]. / Intervalos de referencia poblacional y determinantes de la homocisteína plasmática.

BACKGROUND: There is a well-known relationship between plasma homocysteine levels and the risk of cardiovascular events. Determination of homocysteine levels may also be of potential diagnostic aid in several clinical situations. The construction of reference ranges should take age and sex variations into account. SUBJECTS AND METHODS: 396 healthy subjects were recruited (172 males and 224 females). Fasting plasma homocysteine levels were measured using the fluorescein polarization immunoassay technique (FPIA). Plasma levels of creatinine, folates, vitamin B12 and TSH were measured. 90% reference ranges were estimated by fractional polynomial regression methods. RESULTS: Homocysteine plasma levels ranged from 4.35 micromol/l to 17,71 micromol/l (median 8.62 micromol/l). These concentrations increased with age and were higher in males (median 9.53 micromol/l [range: 5.45-17.5]) than in females (median 7.79 micromol/l[range: 4.35-17.71]). Sex differences decreased in the elderly. Creatinine plasma levels (with a positive association) and folate levels (with a negative association) had a statistically significant effect on the specific distribution of homocysteine levels according to age and sex. CONCLUSIONS: Age-and sex-specific reference ranges of plasma homocysteine have been defined. Renal function along with folate plasma levels have to be accounted for when assessing these distribution ranges.

Indicaciones y limitaciones de la biopsia de la arteria temporal en la arteritis de Horton / Indications and limitations of the biopsy of temporal artery in Horton's arteritis

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[Study of cobalamin deficiency in gastrectomized and aged subjects]. / Estudio del déficit de cobalaminas en sujetos gastrectomizados y de edad avanzada.

OBJECTIVE: The objective of this study were: a) to investigate vitamin B12 deficiency among and advanced aged, clinically healthy, asymptomatic population, presumably with a high prevalence of vitamin B12 deficiency. This deficiency was defined as an increase in the MMA/creatinine ratio in urine and/or basal plasma HT levels which normalize after the administration of vitamin B12; b) its relationship with plasma vitamin B12 levels. SUBJECTS AND METHODS: A total of 45 subjects were studied, 19 in the Group of Gastrectomized patients, who were selected on the basis of an age over 60 years and partial gastrectomy of more than five years, and 26 in the Geriatric Group selected in two nursing homes. All of them were asymptopatic and "healthy" according to data in the clinical records and clinical examination, analytical studies, and none of them was taking any drug that might alter results. After basal analytical studies, which included B12, folates, metilmalonic acid/creatinine (MMA/creatinine) in urine and total plasma homocysteine (TH), i.m. vitamin B12 was administered for 15 days to the gastrectomized patients and oral B12 for 28 days with 2.5 mg folic acid for the last 14 days to the patients in the Geriatric Group. Basal analytical studies were repeated at the end of the study. RESULTS: Basal analytical studies were normal with the exception of two subjects with low levels of hemoglobin and 8 with decreased MCV. Only one subject had decreased serum cobalamines. Nine subjects (20%) were identified with increased basal levels of MMA/creatinine which normalized after decreasing between 97% and 12% after the administration of vitamin B12. TH and MCV also decreased significantly. Basal levels of vitamin B12 ranged from 244 pg/ml to 483 pg/ml (n = 220 pg/ml-980 pg/ml). CONCLUSIONS: Vitamin B12 deficiency, defined as an increase in the MMA/creatinine ratio and TH normalized with B12, is highly prevalent among elderly subjects and may occur in absence of clinical and/or analytical manifestations. Given the widespread recommendation of quantitating MMA and TH for the diagnosis of borderline cases of vitamin B12 deficiency, such disturbance may occur in clinically and biochemically normal subjects. Thus, a special caution should be exerted, given the existing comorbidity in advanced ages, before attributing B12 deficiency to a condition which may be the expression of another concomitant condition.

Estudio del déficit de cobalaminas en sujetos gastrectomizados y de edad avanzada / Study of cobalamine deficiency among advanced aged gastrectomized subjects

Objetivo: El objetivo de este trabajo es investigar en una población de edad avanzada, clínicamente 'sana' y asintomática, en la que se supone una alta prevalencia de carencia de B12, el déficit de esta vitamina definido por un aumento del ácido metilmalónico (AMM)/creatinina en orina y/o de la homocisteína total plasmática (HT) plasmática basal que se normalizan tras la administración de B12, así como su relación con los niveles plasmáticos de dicha vitamina.Sujetos y métodos: Se estudian un total de 45 sujetos pertenecientes 19 al Grupo de Gastrectomizados y seleccionados por ser mayores de 60 años y una gastrectomía parcial de más de 5 años de antigüedad y 26 al Grupo Geriátrico seleccionados en dos residencias geriátricas. Todos estaban asintomáticos y 'sanos' de acuerdo a los datos de la historia y exploración clínica, estudios analíticos, y ninguno tomaba ninguna vitamina ni ningún fármaco que pudiese alterar los resultados. Después de los estudios analíticos basales, que incluían B12, folatos, AMM/creatinina en orina y HT, se administró B12 por vía intramuscular durante 15 días al Grupo de Gastrectomizados y B12 por vía oral durante 28 días con 2,5 mg de ácido fólico durante los últimos 14 días al Grupo Geriátrico. Al finalizar se repitieron los estudios analíticos basales. Resultados: Los estudios analíticos basales fueron normales excepto en dos sujetos con niveles bajos de hemoglobina y ocho con VCM disminuidos. Sólo un sujeto tenía cobalaminas séricas disminuidas. Se identificaron nueve sujetos (20 por ciento) con niveles basales elevados de AMM/creatinina que se normalizaron después de disminuir entre el 97 por ciento y 12 por ciento tras la administración de B12. También descendieron significativamente la HT y el volumen corpuscular medio (VCM). Los valores basales de B12 oscilaban entre 244 y 483 pg/ml (valores normales = 220-980 pg/ml). Conclusiones: La carencia de B12 definida por el aumento del AMM/creatinina y de la HT que se normalizan con B12 tiene una elevada prevalencia en las edades avanzadas y puede ocurrir en ausencia de manifestaciones clínicas y/o analíticas. Dada la recomendación cada vez más extendida de cuantificar el AMM y la HT en el diagnóstico de los casos dudosos de carencia de B12, debe conocerse que dicha alteración puede ocurrir en sujetos clínica y analíticamente normales, lo que aconseja, dada la comorbilidad existente en las edades avanzadas, una especial prudencia antes de atribuir a la carencia de B12 una patología que puede ser la expresión de otra enfermedad concomitante (AU)

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Significado clínico de la homocisteína / Clinical meaning of homocystein

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[Leptospirosis. Review of 11 cases]. / Leptospirosis: Revisión de 11 casos.

Eleven cases of leptospirosis diagnosed from 1988 to 1994 were retrospectively reviewed. The mean age of the patients was 52 years. Epidemiologic factors were found in 10 patients. Hepatorenal involvement was observed in 7 cases (64%), cardiac involvement in 3 (27%), bleeding episodes in 5 (45%) and central nervous system involvement in one case (9%). The Leptospira serogroups identified were: Icterohaemorrhage in 6 cases, Pomona in 1, Sejroe in and could not be determined in 3. One patient died because of multiorgan failure. The epidemiologic, clinical, analytical and therapeutic aspects are discussed.

[Recurrent polychondritis associated with ulcerative colitis]. / Asociación de policondritis recidivante y colitis ulcerosa.

Relapsing polychondritis and Ulcerative Colitis is an uncommon association that has been described only ten times in medical literature. We report a new case of this association in which it was necessary to treat with azathriopine to stop disease progression. Relapsing Polichondritis is a disorder to be considered in patients with Ulcerative Colitis and inflammation of the cartilagenous structures.