Nodular pulmonary light chain deposition disease: an entity associated with Sjögren syndrome or marginal zone lymphoma.

BACKGROUND: Light chain deposition disease (LCDD) is usually a systemic disorder characterised by non-amyloid monoclonal immunoglobulin light chain deposition in tissues. Localised nodular pulmonary (NP) LCDD is a rare and poorly characterised entity and, owing to the difficulties in diagnosis, limited data are available. METHODS: We investigated the clinical, radiological and pathological characteristics of a series of six confidently diagnosed cases of NPLCDD. RESULTS: There were three men and three women with ages ranging from 33 to 74 years. In all cases there were single or multiple pulmonary nodules, in one case associated with cysts. Two patients had no previous history of a lymphoproliferative or autoimmune disorder, two had Sjögren syndrome (SS) and two had extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Lung biopsies led to diagnoses of MALT lymphoma in four patients, including both of those with a previous history of lymphoma and one with SS. In five cases the diagnosis was confirmed by liquid chromatography-tandem mass spectrometry (LC-MS/MS) and in one by electron microscopy. There was no evidence of systemic LCDD in any of the cases. Five patients had an indolent course in spite of limited therapeutic intervention while, in the patient who died, the cause of death was related to the spread of the lymphoma and was not due to the pulmonary lesions. CONCLUSIONS: NPLCDD is an indolent disease, in most cases associated with MALT lymphoma or autoimmune disease.

Follicular helper T-cell lymphoma: a B-cell-rich variant of T-cell lymphoma.

Follicular helper T-cell lymphoma is a recently described variant of T-cell lymphoma sharing the cell of origin with angioimmunoblastic T-cell lymphoma and with primary cutaneous CD4-positive small/medium T-cell lymphoma. To better characterize the morphologic and immunophenotypic features of follicular helper T-cell lymphoma, a series of 4 confidently diagnosed cases are analyzed. The overall morphologic pattern significantly overlaps with that of progressive transformation of germinal centers in 3 cases and with follicular hyperplasia in 1. Detection of large, clustered, atypical T-cells is an important feature differentiating follicular T-cell lymphoma from benign, reactive processes such as progressive transformation of germinal centers. The abnormal T-cells have an immunophenotype identical to that of follicular helper T-cells in all cases. Clonal T-cell populations are detected in all cases. A characteristic setting follicular T-cell lymphomas apart from most other T-cell lymphomas is the abundance of small, mature B-cells. Differences from angioimmunoblastic T-cell lymphoma include the absence of proliferating vessels or of Epstein-Barr virus-positive immunoblasts.

[Late metastases of malignant choroidal melanoma after curietherapy: two case reports]. / Métastases tardives après curiethérapie efficace de mélanome malin de la choroïde. A propos de deux cas.

PURPOSE: The technical advancements now provide conservative treatment of choroidal melanoma. The course of the disease remains marked by the metastases that usually set in within 3 years. The aim of this paper is to report two unusual cases of hepatic metastases of choroidal melanoma that set in 11 and 18 Years after curietherapy. METHODS: A 40-year-old woman and a 50-year-old man presented with T2 and T3 choroidal melanomas and were treated by cobalt plaque. The tumors decreased within the year and the patients were monitored every 3 months for 5 years. RESULTS: Metastases set in, respectively, 11 and 18 years after treatment. DISCUSSION AND CONCLUSION: These two observations show that the metastases had already spread at the moment of diagnosis and that the cell doubling time of the melanoma was very long. These findings indicate the difficulties in monitoring patients with choroidal melanoma.

Genetic forma recombination in permutation flowshop problems.

This paper analyzes different representations for permutation flowshop problems. This is done using forma analysis to assess the quality of these representations with respect to makespan optimization. Classical recombination operators are studied and empirically evaluated in this context. It is shown that the best operators work on representations in which absolute positions of tasks are relevant. Subsequently, some new operators operating on these representations are proposed. These new operators are designed to exhibit specific properties regarding implicit mutation and forma transmission. Their performance is shown to be competitive with traditional operators.

Fluoroscopic coronary artery calcification and associated coronary disease in asymptomatic young men.

Little is known about the diagnostic significance of coronary artery calcification detected fluoroscopically in apparently healthy young men. This study compared the presence of fluoroscopically detected coronary artery calcification with angiographic coronary artery disease in asymptomatic male military aircrew undergoing noninvasive cardiac screening tests and coronary arteriography for occupational indications. Of 1,466 men screened with coronary fluoroscopy, 613 underwent coronary arteriography because of one or more abnormal noninvasive test results. The mean age (+/- SD) of all subjects screened was 40.2 +/- 5 years (range 26 to 65). Significant coronary artery disease (greater than or equal to 50% diameter stenosis) was found in 104 of the 613 subjects with arteriograms (16.9% disease prevalence). Overall sensitivity and specificity for coronary artery calcification detection of significant disease, based only on those subjects undergoing arteriography, were 66.3% and 77.6%, respectively. For measurable disease (mild plus significant), sensitivity was 60.6% and specificity 85.9%. Positive and negative predictive values were 37.7% and 91.9%, respectively, for significant disease. For measurable disease, positive and negative predictive values were 68.9% and 80.9%, respectively. In these asymptomatic young men, a fluoroscopic examination negative for coronary artery calcification indicated a low risk of significant coronary artery disease, whereas a positive test result (calcification present) substantially increased the likelihood of angiographically significant coronary artery disease.

[Efficacy of the electrocardiographic diagnosis of left ventricular hypertrophy on the basis of gender]. / Eficácia do diagnóstico electrocardiográfico de hipertrofia ventricular esquerda segundo o sexo.

STUDY OBJECTIVE: To determine the efficacy of electrocardiographic criteria of Left Ventricular Hypertrophy (RVL greater than 11 mm, SVE3 + RVL greater than 20 mm in females and greater than 28 mm in males, SV1 + RV6 or SV2 + RV5) in hypertensive patients according to sex. DESIGN: Retrospective study. Gold standard M mode echocardiography. SETTING: Pulido Valente Hospital Department of Cardiology (Lisbon). PATIENTS: 56 hypertensive patients (age 55.1 s.d. 11.6), 27 females and 29 males, with LVH by echocardiographic Left Ventricular Mass Index (LVMI) greater than 110 g/m2 in woman and greater than 134 g/m2 in men, respectively 10 and 19 patients. The LVMI was calculated by the formula of the American Society of Echocardiography modified by Devereux. RESULTS: The LVMI was smaller in females than in males (P = 0.007). The Cornell Criteria had the greatest efficacy in woman and the Sokolow criteria satisfactory efficacy but in man. The electrocardiogram (1 of the 4 criteria positive) had low diagnostic efficacy in woman. CONCLUSIONS: We concluded that echocardiogram is the best non-invasive diagnostic tool of Left Ventricular Hypertrophy in hypertensive females and is necessary to adjust and choose the electrocardiographic Left Ventricular Hypertrophy criteria according to sex.

[Arrhythmia in chronic respiratory insufficiency]. / Disritmias em insuficientes respiratórios crónicos.

Ventricular arrhythmias are frequent in chronic pulmonary disease. Hypoxemia, right ventricular enlargement, associated left ventricular dysfunction and iatrogenic factors are potential determinants of the density and complexity of these arrhythmias. Its prognostic meaning in chronic pulmonary disease and its relationship with the elevated prevalence of sudden cardiac death in these patients, are not well known. Meanwhile, the therapeutic anti-arrhythmic strategy is dominated by the correction of the etiologic factors, namely continuous oxygen therapy which decreases ventricular arrhythmia and prolongs survival. Holter 24 hours monitoring is a useful diagnostic tool in management of these patients.