Early Onset of HTLV-1 Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) and Adult T-cell Leukemia/Lymphoma (ATL): Systematic Search and Review.

Human T-cell lymphotropic virus type 1 (HTLV-1) is endemic in some regions and its vertical transmission occurs mainly through breastfeeding. About 10% of carriers develop associated diseases including HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP), adult T-cell leukemia/lymphoma (ATL) and infectious dermatitis associated with HTLV-1 (IDH). We searched for available case reports of early-onset HAM/TSP and ATL to evaluate demographic and disease aspects in infantile-juvenile patients. In the reviewed literature, 27 HAM/TSP and 31 ATL cases were found. In almost all of them, the most likely route of transmission was through breastfeeding. ATL is rarely reported, notwithstanding it may be underestimated because T-cell lymphomas are not investigated for HTLV-1 infection in this age group. IDH was frequently associated with HAM/TSP. The investigation of HTLV-1 infection in pregnant women is an important matter of public health and should be mandatory in endemic countries.

HTLV-1 proviral load in infective dermatitis associated with HTLV-1 does not increase after the development of HTLV-1-associated myelopathy/tropical spastic paraparesis and does not decrease after IDH remission.

INTRODUCTION: Infective dermatitis associated with HTLV-1 (IDH) is a recurrent eczema which affects children vertically infected with HTLV-1. In Bahia, Brazil, we recently reported that 47% of IDH patients also develop juvenile HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP), a progressive disabling disorder which is typically reported in adult HTLV-1 carriers. IDH may also predispose to adult T-cell leukemia/lymphoma, a neoplasm associated with HTLV-1. The factors relating to the development of HTLV-1-associated juvenile diseases have not yet been defined. HTLV-1 proviral load (PVL) is one of the main parameters related to the development of HTLV-1 associated diseases in adults. In the current study, we investigated the role of PVL in IDH and juvenile HAM/TSP. METHODOLOGY/PRINCIPAL FINDINGS: This is a cohort study that included fifty-nine HTLV-1 infected children and adolescents, comprising 16 asymptomatic carriers, 18 IDH patients, 20 patients with IDH and HAM/TSP (IDH/HAM/TSP) and five with HAM/TSP. These patients were followed-up for up to 14 years (median of 8 years). We found that PVL in IDH and IDH/HAM/TSP patients were similarly higher than PVL in juvenile asymptomatic carriers (p<0.0001). In those IDH patients who developed HAM/TSP during follow-up, PVL levels did not vary significantly. HAM/TSP development did not occur in those IDH patients who presented high levels of PVL. IDH remission was associated with an increase of PVL. Inter-individual differences in PVL were observed within all groups. However, intra-individual PVL did not fluctuate significantly during follow-up. CONCLUSIONS/SIGNIFICANCE: High PVL in IDH patients was not necessary indicative of progression to HAM/TSP. PVL did not decrease after IDH remission. The maintenance of high PVL after remission could favor early development of ATL. Therefore, IDH patients would have to be followed-up even after remission of IDH and for a long period of time.

Adult T-cell leukemia/lymphoma in South and Central America and the Caribbean: systematic search and review.

Adult T-cell leukemia/lymphoma (ATL) is caused by the human T-cell lymphotropic virus type 1 (HTLV-1) which is endemic in countries of Caribbean and Central and South America. We performed a systematic search and review to identify publications on ATL in these countries to verify if this disease was getting recognition in these regions as well as the characteristics of the observed cases. The median age of 49.4 years was lower than that referred to in Japan. According to our findings in most Brazilian states and in some other countries, ATL is not being recognized and should be strongly considered in the differential diagnosis of T-cell leukemias/lymphomas. Failure to identify these cases may be due to the unsystematic realization of serology for HTLV-1 and phenotypic identification of non-Hodgkin lymphomas that may result from lack of resources. Detection of ATL cases has been more feasible with cooperation from foreign research centers. A huge effort should be made to improve the surveillance system for ATL diagnosis in most of the South- and Central-American and Caribbean countries, and this attitude should be embraced by public organs to support health professionals in this important task.

Chemical interesterification of blends with palm stearin and patawa oil.

The present study sought to develop lipid bases from blends between patawa oil and palm stearin. These blends were analyzed before and after the chemical interesterification process for their fatty acid and triacylglycerol composition, free fatty acid (FFA) content, peroxide index, thermal properties, melting point, consistency, and solid fat content (SFC). Blends with unsaturated fatty acid contents between 60 and 70% were obtained, with a good ratio between saturated and unsaturated fatty acids, which indicates a healthy content of fatty acids. Variations in the triacylglycerol contents and melting and crystallization thermograms evidenced the reaction. The blend with 50% stearin and 50% patawa oil showed the best results after the chemical interesterification reaction regarding the possible application in fatty products for its appropriate melting point, SFC similar to that of soft table margarines, plastic and spreadable consistency at refrigeration temperature, thus combining physical and nutritional properties desirable for the food industry.

Laparoscopic gastric banding in the rat model as a means of videolaparoscopic training.

BACKGROUND: The development of laparoscopy in bariatric surgery has attracted a large number of surgeons. Learning this method for future clinical practice requires intensive training with inert tissues, simulators and experimental surgery in animals. Performing these procedures in small animals, with the same equipment used in humans, is feasible, allowing familiarization with and comprehension of the basic techniques. Wistar rats weighing 300-600 g were used. The animals were kept in standard laboratory conditions. A laparoscopic video-system, Veress needle, three ports, a 0 degree optic, a laparoscopic needle-holder, two 5-mm graspers, a 5-mm dissection clamp and a 5-mm scissors were used. An orogastric catheter with three 4-0 nylon sutures and one 6-0 nylon suture were also utilized. For the gastric band, we used a plastic device similar to the human gastric band. The present study describes a simple, inexpensive and reproducible technique for laparoscopic gastric banding in a rat model utilizing the same instruments developed for humans. The experimental rat model is more motivating than simulators, requires less space, and has easier maintenance compared with bigger animals, and consequently allows the use of more animals for teaching, training and application in many scientific studies.

Correlations between clinical and pathological features in 17 cases of mycosis fungoides before and after transformation.

BACKGROUND: Mycosis fungoides (MF) may progress to transformed MF (T-MF), a condition with aggressive behavior. OBJECTIVES: This study was designed to compare the clinical and pathological features of biopsies in 17 cases of MF before and after transformation. METHODS: During a revision of primary cutaneous T cell lymphomas, 53 cases of MF were identified, including 17 cases of T-MF. Clinical, pathological, and immunohistochemical data for the MF patients were evaluated. Cases of T-MF and intermediate transformed (IT) MF were diagnosed according to previous criteria. The histological and immunohistochemical features of T-MF biopsies were compared with those of MF/IT-MF biopsies taken before or concomitant with transformation. RESULTS: At the initial diagnosis, three patients were found to have more advanced stages of disease: two had MF and T-MF simultaneously, and another had only oral T-MF. Four patients considered to show histological transformation maintained disease stages Ia and Ib and all remain alive. Of five patients with IT-MF at first diagnosis, all progressed to complete histological transformation, three developed tumors, and two died of disease. Four patients progressed to CD30+ large cell lymphoma, and three of these died of disease. In one of these patients, the MF biopsy showed a high level of expression of CD30 in the epidermis and dermis. CONCLUSIONS: No correlation between advanced MF and expression of CD25 and CD30, or frequency of Ki-67+ cells was found. The frequency of transformation among patients with initially non-transformed MF was high. Our findings support the emphasis given by other authors to IT-MF, a pattern of MF which is generally not considered in many studies.

Microsatellite alterations are also present in the less aggressive types of adult T-cell leukemia-lymphoma.

BACKGROUND: Adult T-cell leukemia/lymphoma (ATL) is a mature T-cell neoplasia etiologically linked to HTLV-1. Manifestations of ATL are diverse and different clinical types with different tissue involvement and aggressiveness have been described. The mechanisms that lead to the development of ATL clinical types have not yet been clarified. Considering that in ATL patients HTLV-1 infection generally occurs in childhood, a multistep carcinogenesis model has been proposed. Microsatellite alterations are important genetic events in cancer development and these alterations have been reported in the aggressive types of ATL. Little is known about oncogenesis of the less aggressive types. METHODOLOGY/PRINCIPAL FINDINGS: In this study we investigated the role of the microsatellite alterations in the pathogenesis mediated by HTLV-1 in the different types of ATL. We examined the presence of microsatellite instability (MSI) and loss of heterozigosity (LOH) in matched pair samples (tumoral and normal) of 24 patients with less aggressive types (smoldering and chronic) and in aggressive types (acute and lymphoma) of ATL. Four microsatellite markers D10S190, D10S191, D1391 and DCC were analyzed. MSI was found in four patients, three smoldering and one chronic, and LOH in four patients, three smoldering and one acute. None of the smoldering patients with microsatellite alterations progressed to aggressive ATL. CONCLUSIONS/SIGNIFICANCE: To our knowledge, this is the first report describing the presence of MSI and LOH in the less aggressive types of ATL. These results indicate that microsatellite alterations may participate in the development of the less aggressive types of ATL.

Double integration band of HTLV-1 in a young patient with infective dermatitis who developed an acute form of adult T-cell leukemia/lymphoma.

Few cases of acute adult T-cell leukemia/lymphoma (ATL) have been diagnosed in young patients. This report is the first to describe a young girl with infective dermatitis associated with HTLV-1 that progressed to acute ATL with Southern blot hybridization and gamma-TCR-rearrangement revealing a monoclonal pattern with two copies of the provirus.

Adult T-cell leukemia/lymphoma triggered by adalimumab.

Here, we describe a 48-year-old woman infected by the human T-cell lymphotropic virus type 1 (HTLV-1) with spondyloarthritis, uveitis, bilateral episcleritis and neurogenic bladder. She had a history of a probable infective dermatitis associated with HTLV-1 (IDH) in childhood. After the use of adalimumab, she developed lymphocytosis and a cutaneous lymphoma associated with IDH. She had the diagnoses of IDH and of chronic adult T-cell leukemia/lymphoma, supported by the demonstration of proviral integration in the cutaneous lesion.

Analysis of cutaneous lymphomas in a medical center in Bahia, Brazil.

OBJECTIVES: To evaluate the frequency of the different types of cutaneous lymphoma (CL) in 1 university hospital in Brazil and compare this frequency with those observed in other countries. METHODS: After review, 72 (84.7%) cases of primary cutaneous T-cell lymphoma (CTCL) and 13 (15.3%) cases of primary cutaneous B-cell lymphoma (CBCL) were included. RESULTS: Of the CTCLs, 40.3% were mycosis fungoides (MF); 26.4% were adult T-cell leukemias/lymphomas (ATLs); 23.6% were peripheral T-cell lymphomas, unspecified; and 8.3% were anaplastic large cell lymphomas. Of the MF cases, 17.2% progressed to transformed MF. Five-year survival for primary human T-cell lymphotropic virus type 1-negative CTCL, ATL, and CBCL was 64.0%, 42.1%, and 62.5%, respectively. MF and ATL were the most frequent primary CTCLs. CONCLUSIONS: The frequencies observed here are close to those observed in Peru but different from those of European countries. Unfortunately, the World Health Organization/European Organization of Research and Treatment of Cancer classification does not include primary cutaneous ATL.