[Pulmonary Langerhans'-cell histiocytosis]. / Histiocitosis pulmonar de células de Langerhans.

Pulmonary Langerhans'-cell histiocytosis belongs to a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by Langerhans' cells. It is an uncommon interstitial lung disease of unknown etiology occurring almost exclusively in cigarette smokers. It s course in adults is variable and unpredictable, ranging from benign self-limiting types with spontaneous regression to slowly progressive malignant disease that leads to respiratory failure and death. We report one patient diagnoses of pulmonary Langerhans' cell histiocytosis who experimented an objective radiographic improvement and disappearance of symptoms after smoking cessation.

Histiocitosis pulmonar de células de Langerhans / Pulmonary Langerhans´-cell histiocytosis

La histiocitosis pulmonar de células de Langerhans forma parte de un espectro de enfermedades caracterizadas por la proliferación monoclonal y la infiltración de distintos órganos por células de Langerhans. Es una enfermedad pulmonar intersticial de etiología desconocida que ocurre casi exclusivamente en pacientes fumadores. El curso de esta enfermedad en adultos es impredecible, oscilando desde formas benignas autolimitadas, hasta formas malignas con evolución progresiva hacia el fallo respiratorio y la muerte. Presentamos el caso de un paciente diagnosticado de histiocitosis pulmonar de células de Langerhans que presentó una mejoría clínica y radiográfica tras abandonar el hábito tabáquico

Pulmonary Langerhans´- cell histiocytosis belongs to a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by Langerhans´cells. It is an uncommon interstitial lung disease of unknown etiology occurring almost exclusively in cigarette smokers. It´s course in adults is variable and unpredictable, ranging from benign self-limiting types with spontaneous regression to slowly progressive malignant disease that leads to respiratory failure and death. We report one patient diagnoses of pulmonary Langerhans´cell histiocytosis who experimented an objetive radiographic improvement and disappearance of symptoms after smoking cessation

Differential expression of NF-kappaB pathway genes among peripheral T-cell lymphomas.

Nuclear factor kappa B (NF-kappaB) is one important pathway in T-cell proliferation and survival. In a previously reported microarray study, we found NF-kappaB pathway genes differentially expressed between peripheral (PTCL) and lymphoblastic lymphomas. Here, we investigated the expression of NF-kappaB pathway genes using cDNA microarrays in a group of 62 PTCL and in reactive lymph nodes. We found two different subgroups of PTCL based on the expression of NF-kappaB pathway genes. One-third of PTCL showed clearly reduced expression of NF-kappaB genes, while the other group was characterized by high expression of these genes. This distinction was found among all T-cell lymphoma categories analyzed (PTCL unspecified, angioimmunoblastic, cutaneous and natural killer/T lymphomas) with the exception of anaplastic lymphomas (ALCL), which were characterized by reduced NF-kappaB expression in anaplastic cells. Quantitative RT-PCR and immunohistochemical analysis of NF-kappaB-p65 protein confirmed these differences among PTCL subgroups. Importantly, we found that differentiation between NF-kappaB-positive and -negative PTCL could be of clinical interest. The expression profile associated to reduced expression of NF-kappaB genes was significantly associated with shorter survival of patients and seems to be an independent prognostic factor in a multivariate analysis.

[Whipple's disease. Two new cases of an underdiagnosed disease]. / Enfermedad de Whipple. Dos nuevos casos de una enfermedad infradiagnosticada.

Whipple's disease is a rare systemic infectious disease caused by the bacterium Tropheryma whippelii. Early diagnosis is essential. Whipple's disease is potentially fatal but responds dramatically to antibiotic treatment. The diagnosis is confirmed by means of polymerase chain reaction (PCR) technology. This analysis may be useful for monitoring the efficacy of therapy. The recommended treatment al present is administration of cotrimoxazole twice daily for one year. When CNS involvement occurs, it is recommended initial treatment with daily parenteral administration of streptomycin 1 g and 1.2 million units of benzyl penicillin (Penicillin G) over a period of 14 days.

Enfermedad de Whipple. Dos nuevos casos de una enfermedad infradiagnosticada / Whipple´s disease. Two new cases of an underdiagnosed disease

La enfermedad de Whipple, también conocida como lipodistrofia intestinal, es un proceso infeccioso, multisistémico y poco frecuente, causado por la bacteria Tropheryma whippelii. El diagnóstico precoz es fundamental porque esta enfermedad es potencialmente letal, pero responde espectacularmente al tratamiento antibiótico. El diagnóstico se confirma con la reacción en cadena de la polimerasa (PCR) que también es útil para monitorizar la respuesta al tratamiento. Actualmente el tratamiento recomendado consiste en la administración de cotrimoxazol oral dos veces al día durante un año. Cuando hay afectación del SNC se aconseja iniciar el tratamiento con la administración intramuscular de 1 gramo de estreptomicina asociado a 1,2 MU de bencil penicilina (penicilina G) de forma diaria durante 14 días

Whipple’s disease is a rare systemic infectious disease caused by the bacterium Tropheryma whippelii. Early diagnosis is essential. Whipple’s disease is potentially fatal but responds dramatically to antibiotic treatment. The diagnosis is confirmed by means of polymerase chain reaction (PCR) technology. This analysis may be useful for monitoring the efficacy of therapy. The recommended treatment al present is administration of cotrimoxazole twice daily for one year. When CNS involvement occurs, it is recommended initial treatment with daily parenteral administration of streptomycin 1 g and 1,2 million units of benzyl penicillin (Penicillin G) over a period of 14 days

Obstrucción de la arteria pulmonar / Pulmonary artery sarcoma

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Eje hipófiso-suprarrenal en el alcoholismo crónico / Hypophyso-adrenal axis in patients with chronic alcoholism

Objetivos: Valorar la situación del eje hipófiso-suprarrenal en pacientes con alcoholismo crónico. Método: Se estudiaron 24 pacientes alcohólicos crónicos ingresados en nuestro centro en situación de delirio agudo. Se determinaron cortisolemia a.m. y p.m. y niveles de ACTH a.m.junto a serie roja con sus índices de Wintrobe, número de plaquetas, trigliceridemia, colesterolemia total y HDL y actividad de transaminasas, fosfatasa alcalina y gammaglutamiltranspeptidasa en el suero. Resultados: Las cifras medias de ACTH a.m. fueron de 41.2 pcg/ml para valores normales de 10-80 pcg/ml, las de cortisolemia a.m. de 22 mcg/ml (rango normal 5-25 mcg/ml) y las de cortisolemia p.m. de 12.3 mcg/ml (tango normal 5-15 mcg/ml). Conclusiones: Las cantidades de alcohol ingeridas por los pacientes alcohólicos no parecen equiparables a las utilizadas y a las que condicionan una hiperfunción suprarrenal en la experimentación animal. Los niveles de ACTH en sangre y de cortisolemia no son útiles como parámetros indirectos de alcoholismo. Al respecto, destacan como más efectivos los índices de Wintrobe y la actividad de gammaglutamiltranspeptidasa en el suero. (AU)

[Rhabdomyolysis syndrome and hyperthermia secondary to cocaine and/or ecstasy consumption. Report of 2 new cases and review of the problem]. / Síndrome de rabdomiolisis e hipertermia secundario al consumo de cocaína y/o éxtasis. Comunicación de dos nuevos casos y revisión del problema.

We describe two cases of hyperthermia and rhabdomyolysis related to cocaine and/or ecstasy consumption. The clinical and laboratory findings are characteristic of hyperthermia syndrome in both cases. The first patient consumed cocaine, ecstasy and alcohol and afterwards suffers from hyperthermia and rhabdomyolysis associated with decreased conscious level, labile blood pressure, severe renal insufficiency (needing hemodialysis) and important liver failure. The second patient consumed ecstasy and alcohol with intense physical activity and shows the same but moderate clinical picture rapidly responding to intravenous fluid therapy. The incidence of this drug-induced hyperthermia is low compared to the important use of these illicit drugs. Nevertheless its increasing consumption will lead physicians to attend more patients with this syndrome and other related complications. For this reason we have reviewed and up-dated this problematic subject.

Síndrome de rabdomiolisis e hipertermia secundario al consumo de cocaína y/o éxtasis. Comunicación de dos nuevos casos y revisión del problema / Rhabdomyolisis syndrome and hyperthermia secondary to cocaine and/or ecstasy intake. Communication of two new cases and review of the problem

Se describen dos casos de síndrome de hipertermia y rabdomiolisis en pacientes que habían consumido cocaína y/o éxtasis. Ambos muestran una clínica y unas alteraciones analíticas características de este síndrome.El primer paciente a consecuencia de la toma de cocaína, éxtasis y alcohol sufre un cuadro grave de hipertermia y rabdomiolisis asociado a estupor, confusión, tensión arterial lábil, grave insuficiencia renal, que precisó de hemodiálisis, e intenso daño hepático. El segundo consume éxtasis asociado a alcohol e importante ejercicio físico y sufre de un cuadro similar aunque moderado que revirtió con medidas de reposición hidrolectrolítica. Si bien la incidencia de esta patología es muy limitada en relación al amplísimo consumo de estas drogas, su continuo uso llevará consigo la atención de nuevos pacientes afectos de esta y otras complicaciones agudas. Con este motivo se realiza una revisión y actualización del problema (AU)

[Hypophysio-adrenal axis in patients with chronic alcoholism]. / Eje hipófiso-suprarrenal en el alcoholismo crónico.

OBJECTIVES: To study the situation of the hypophyso-adrenal axis in patients with chronic alcoholism. METHOD: 24 patients with chronic alcoholism admitted in our Institution with acute delirium were studied. Cortisol am and pm und (ACTH levels, along with hemogram with Wintrobe indexes, patelets number, triglicerides, total and HDL cholesterol, transaminases, alkaline phosphatase and gammaglutamyltranspeptidase were measured in serum. RESULTS: The mean values of ACTH am were 41.2 pcg/ml (normal values 10-80 pcg/ml), of plasma cortisol am 22 mcg/ml (normal values 10-24 mcg/ml) and plasmo cortisol pm 12.3 mcg/ml (normal values 5-12 mcg/ml). CONCLUSIONS: The amount of alcohol consumed by alcoholic patients ist not comparable to that used in animal models, which induced adrenal hyperfunction. Blood ACTH and cortisol levels are not useful as indirect markers of alcoholism. The Wintrobe indexes and serum gammaglutamyltranspeptidase levels are more useful for that purpose.

[Postsurgical meningitis caused by Pseudomonas aeruginosa: study of 15 cases and review of the literature]. / Meningitis postquirúrgica por Pseudomonas aeruginosa: estudio de 15 casos y revisión de la literatura.

OBJECTIVE: Pseudomonas aeruginosa meningitis is a rare condition which is usually associated with pathology in the ORL field, neurosurgery or local neurologic manipulations. The characteristics, epidemiology, and course of this entity were determined. METHODS: Fifteen episodes of nosocomial postsurgical Pseudomonas aeruginosa meningitis occurred between 1989 and 1996 were retrospectively analyzed. RESULTS: A previous cranioencephalic trauma was recorded in 46.6% of patients. The portals of entry included: intraventricular catheter (IC) (12 cases), CSF fistula (2 cases), and craniotomy (1 case). In five occasions (41.6%) the microorganism was also recovered from the intraventricular catheter. Once culture results were available, therapy with active drugs against Pseudomonas was instituted and in 7 occasions was accompanied by the removal of IC. Eight patients eventually cured and two patients relapsed. The absence of cure was significantly associated with non-removal of the IC (p < 0.01). The infection resulted in death in 26.6% of patients. CONCLUSIONS: Postsurgical Pseudomonas aeruginosa meningitis is an entity of growing relevance. It is associated with relevant morbi-mortality. Catheter removal is essential to obtain a favorable outcome.

Meningitis postquirúrgica por Pseudomonas aeruginosa: estudio de 15 casos y revisión de la literatura / Postsurgical Pseudomonas aeruginosa meningitis: report of 15 cases and review of the literature

Objetivo. La meningitis por Pseudomonas aeruginosa es una entidad poco frecuente que suelen asociarse a patología del área otorrinolaringológica, intervenciones neuroquirúrgicas o manipulaciones neurológicas locales. Se determinaron las características, epidemiología y evolución de esta entidad.Métodos. Se analizaron de forma retrospectiva entre 1989-1996 quince episodios de meningitis nosocomial postquirúrgica por Pseudomonas aeruginosa. Resultados. En un 46,6 por ciento de los pacientes se recogió el antecedente de un traumatismo craneoencefálico.Las puertas de entrada fueron: catéter intraventricular (doce casos), en dos una fístula de líquido cefalorraquídeo y en uno la craniotomía. En cinco ocasiones (41,6 por ciento) el microorganismo se cultivó también en el catéter intraventricular. Tras la llegada del cultivo se instauró tratamiento con fármacos activos frente a Pseudomonas, que en siete ocasiones se acompañó de la retirada del catéter intraventricular. En ocho casos se produjo la curación y dos enfermos recidivaron. La ausencia de curación se asoció de forma significativa (p < 0,01) a la no retirada del catéter. El 26,6 por ciento de los pacientes falleció a consecuencia de la infección. Conclusiones. La meningitis postquirúgica por Pseudomonas aeruginosa es una entidad de importancia creciente. Se asocia a una importante morbimortalidad. La retirada del catéter es fundamental en su curación (AU)

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Other cancers in patients with gastric MALT lymphoma.

Patients with Hodgkin's disease and nodal non-Hodgkin's lymphomas seem to have an excess risk for other cancers. A high incidence of other cancers has also been found in some series of patients with gastric MALT lymphomas. In a series of 136 patients with gastric MALT lymphomas the occurrence and features of other cancers have been described. In order to evaluate their occurrence statistically (excluding skin cancers) standard incidence ratios (SRI) have been calculated, using the incidence rates of a Cancer Registry in Spain as a reference. A Cox's multivariate proportional hazard model was fitted in order to evaluate the influence of age, sex, histological grade and treatment with chemotherapy or chemotherapy plus radiotherapy in the development of other non-skin cancers occurring after the diagnosis of MALT lymphoma. Other cancers were detected in 16 of the 136 patients (11.7%); the other cancer was detected prior to MALT gastric lymphoma in 6 patients (4.41%), concomitantly in 4 (2.9%) and after diagnosis of the lymphoma in 6 (4.41%). Other cancers occurred in 14.4% of the male and in 8.3% of the female patients; in 12% of the patients with low grade and in 11% of the patients with high grade lymphomas. Of the 6 cancers that occurred after diagnosis of the gastric lymphoma, 3 did in the 80 patients (3.7%) that had been treated with chemotherapy, 1 in the 3 cases (33%) treated with chemotherapy and radiotherapy and 2 in the 53 patients (3.7%) who had not received chemotherapy or radiotherapy. The most frequent other cancers were lymphoid neoplasms and gastric carcinoma. There was not an excess of other cancers in the whole cohort or in the sex or histological grade strata. There was an excess close to significance (SIR =2.59; 95% CI:0.98-6.88) in the patients under 50 years of age. In the Cox's analysis, age, sex, histological grade and treatment did not influence the occurrence of other cancers after the diagnosis of lymphoma. In conclusion, in patients with gastric MALT lymphoma other cancers also occur. An excess incidence was not demonstrated, although it may exist in patients under 50 years. Of special importance is the occurrence of gastric cancer that appears concomitantly or after gastric lymphoma.

Peripheral T-cell lymphomas: initial features, natural history, and prognostic factors in a series of 174 patients diagnosed according to the R.E.A.L. Classification.

BACKGROUND: Peripheral T-cell lymphomas (PTCL) account for about 10% of all lymphomas in Western countries. The aim of the present study is to analyze the initial characteristics and prognostic factors in a large series of PTCL patients. PATIENTS AND METHODS: 174 patients (105 male/69 female; median age 61 years) were diagnosed with PTCL according to the R.E.A.L. Classification in nine Spanish institutions between 1985 and 1996. Cutaneous lymphomas and T-cell chronic lymphocytic/prolymphocytic leukemia were excluded from the study. Univariate and multivariate analyses were used to assess the prognostic value of the main initial variables. RESULTS: The distribution according to histology subgroup was: PTCL unspecified, 95 cases (54.4%); anaplastic large-cell Ki-l-positive (ALCL), 30 cases (17%); angioimmunoblastic T cell, 22 cases (13%); angiocentric, 14 cases (8%); intestinal T cell, 12 cases (7%), and hepatosplenic gamma delta T cell, one case (0.6%). As compared to the other types, ALCL presented more frequently in ambulatory performance status, without extranodal involvement, in early stage, normal serum beta 2-microglobulin (B2M) level and low-risk international prognostic index (IPI). Most patients were treated with adriamycin-containing regimens. The overall CR rate was 49% (69% for ALCL vs. 45% for other PTCL; P < 0.02). The risk of relapse was 48% at four years. Median survival of the series was 22 months (65 months for ALCL vs. 20 months for other PTCL; P = 0.03), with a four-year probability of survival of 38% (95% confidence intervals (95% CI): 28-48). In the univariate analysis, in addition to the histology, older age, poor performance status, presence of B-symptoms, extranodal involvement, bone marrow infiltration, advanced Ann Arbor stage, high serum LDH, high serum B2M, and intermediate- or high-risk IPI were related to poor survival. In the multivariate analysis the histologic subgroup (ALCL vs. other PTCL) (P = 0.02; response rate (RR): 4.3), the presence of B-symptoms (P = 0.02, RR: 2.2), and the IPI (low vs. high) (P = 0.04, RR: 2) maintained independent predictive value. When the analysis was restricted to the unspecified subtype, only IPI had independent prognostic value (P = 0.003; RR: 3.5). CONCLUSIONS: PTCL have adverse prognostic features at diagnosis, respond poorly to therapy and have short survival, with no sustained remission. ALCL constitutes a subgroup which responds better to therapy and has a longer survival.

[Pyogenic hepatic abscess. Review of 59 cases and experience with imipenem]. / Abscesos hepáticos piogénicos. Revisión de 59 casos y experiencia con imipenem.

OBJECTIVES: To study the different etiopathogenic, microbiological, clinical, evolutive, and therapeutic aspects in patients with pyogenic liver abscesses, with a special emphasis in the usefulness of imipenem-cilastatin therapy. MATERIALS AND METHODS: The clinical records of 59 patients with liver abscesses (45 single abscess and 14 multiple abscesses) diagnosed at our institution in the last eleven years were studied. RESULTS: The most common predisposing conditions included biliary (35.6%) and colon (15.3%) diseases, and abdominal trauma (15.3%). The microorganisms responsible for these abscesses included E. coli, Bacteroides spp., and different streptococci. CT and/or abdominal echography were the diagnostic techniques most commonly used. Twenty-three patients were treated with percutaneous drainage and antibiotics, 22 with surgical drainage and antibiotics, 6 with both types of drainage and antibiotics, and 8 exclusively with antibiotics. Twenty-three patients received imipenem (1 g/IV/8 h) and 29 other antibiotics. Twelve patients died and 9 required admission at the ICU. With regard to patients treated with imipenem, 17 (73.9%) cured, 3 of them (one single abscess and two multiple abscesses) without drainage. Two patients treated with imipenem (8.7%) and 4 treated with other antibiotics (13.8%) relapsed. CONCLUSIONS: Imipenem can be a useful antibiotic in association with percutaneous or surgical drainage for the treatment of pyogenic liver abscesses.

Evaluation of the international index in the prognosis of high grade gastric malt lymphoma.

The International Prognostic Index identifies four risk groups with different survival rates in aggressive non-Hodgkin's lymphoma. We have studied whether a slight modification of this index has prognostic significance in high grade gastric B-cell MALT lymphoma. In 53 patients with high grade gastric B-cell MALT lymphoma the following survival factors were investigated: age over or under 60 years, sex, B symptoms, more than one extranodal site of involvement other than the stomach, serum LDH levels, performance status, stage I/IIE1/IIE2 v.s. stage III/IV, treatment with surgery, chemotherapy or both modalities together and the four risk groups as defined by the Modified International Prognostic Index (MIPI). A multivariate Cox's test was used to evaluate the independent prognostic significance on survival of all the above variables. Advanced stage (III/IV) and involvement of more than one extranodal site not including stomach were the only variables influencing survival. The MIPI was not sufficient to separate groups with significant differences in survival or to stratify prognostic groups. In this series, the MIPI did not show prognostic significance in high grade gastric B-cell MALT lymphoma.

Gastric B-cell mucosa-associated lymphoid tissue (MALT) lymphoma. Clinicopathological study and evaluation of the prognostic factors in 143 patients.

BACKGROUND: Gastric MALT lymphoma can be histologically classified into two groups, low-grade (LG) and high-grade (HG); however, their natural history is poorly understood. We have studied a large retrospective series aiming to confirm whether the histological groups confer different clinical features and behavior and to analyze the prognostic factors in these patients. PATIENTS AND METHODS: A series of 143 gastric B-cell MALT lymphomas is reported. Eighty-four were low-grade lymphomas (LG) and 59 were high-grade lymphomas (HG). Median follow-up was 36 months. The clinical and analytical parameters of the 84 LG patients were compared with those of the 59 HG patients. In the patients who had been operated on, the pathological features (macroscopical patterns, tumor size, involvement of resection margins, degree of parietal invasion and involvement of abdominal lymph nodes and adjacent viscera) of the LG patients were compared with those of the HG patients. The sites of relapses were studied. In the 132 treated and followed-up patients the influence of the treatment and that of clinical, analytical and pathological features on survival were investigated with the Kaplan and Meier and log-rank tests. To identify the factors with independent influence on survival, a Cox model was fitted for the whole series and separately for 53 HG patients. RESULTS: HG group differed from the LG group by a significantly higher frequency of weight loss at presentation, palpable abdominal mass, hepatomegaly, peripheral lymphadenopathy, elevated serum LDH, higher incidence of stage III-IV and tumor/mass patterns in the endoscopy and in the gastrectomy specimen. The tumor was significantly larger in the HG group than in the LG and the deeper invasion of the gastric wall, the higher frequency of infiltration of the abdominal lymph nodes and the visceral extension were also significant in the HG group. Complete remission (CR) was achieved in 91% of the patients of the LG group, but was significantly lower, 70%, in the HG group. Relapses occurred in the stomach and also in non-MALT sites. In 132 treated and followed-up patients, elevated serum LDH, absence of CR, HG group and stage III-IV were associated with a worse survival. In the Cox multivariate model, stage was the only variable influencing survival, although stage was related to the histological grade. In the HG group, stage was also an independent significant risk factor, whereas treatment with surgery, chemotherapy or both was not. In the 103 patients treated with surgery, a worse survival was associated with the involvement of the resection borders, depth of the infiltration of the gastric wall, dissemination to distant abdominal nodes and adjacent organs, but not with the addition of chemotherapy. CONCLUSIONS: Histological classification into LG and HG separates distinctive groups of gastric MALT lymphoma that show striking clinical and prognostic differences. Besides histological grade, stage is the most important prognostic feature.