RESUMEN

Reportamos el caso de una paciente con un cuadro grave de falla multisistémica en la cual se detectaron cambios cromosómicos masivos en estado de mosaico, que se consideran estarían asociados a la posible patología de fondo: un síndrome linfoproliferativo de Células T. La paciente también presentaba un cuadro de Eritrodermia de causa desconocida instaurado varios años antes, con un declive progresivo de su condición física.  El espectro de las manifestaciones clínicas se presenta entonces como un cuadro cutáneo inespecífico paraneoplásico de varios años de evolución y hallazgos de cromoanagénesis en la última fase de un proceso mórbido linfoproliferativo. Las nuevas técnicas de análisis genético permiten obtener datos para aclarar los procesos diagnósticos e identificar una desorganización grave de la información genética que se considera responsable de cambios neoplásicos. (provisto por Infomedic International)

We report the case of a patient with severe multisystemic failure in which massive chromosomal changes in mosaic state were detected, which are considered to be associated with the possible underlying pathology: a T-cell lymphoproliferative syndrome. The patient also presented a case of Erythroderma of unknown cause several years earlier, with a progressive decline in her physical condition. The spectrum of clinical manifestations is then presented as a nonspecific paraneoplastic cutaneous picture of several years of evolution and findings of chromoanagenesis in the last phase of a lymphoproliferative morbid process. The new techniques of genetic analysis make it possible to obtain data to clarify the diagnostic processes and to identify a serious disorganization of the genetic information considered to be responsible for neoplastic changes. (provided by Infomedic International)

RESUMEN

INTRODUCTION: Latin American countries (LATAMC) represent a large fraction of patients treated for multiple myeloma (MM) worldwide. In order to understand the difficulty of access to anti-myeloma therapy in LATAMC, we designed this study that explores areas involved in the availability of drugs, such as health care systems, approval times, coverage of new agents, old drugs, use of generics, and the first-line treatments. MATERIAL AND METHODS: We collected data from 16 countries in 2015. RESULTS: The majority of LATAMC (88%; n = 14) had mixed public and private coverage, with patients with MM cared for in public institutions. Although bortezomib and lenalidomide were approved in 100% and 73% in LATAMC, these figures did not translate to real-world practice as one-half of the nations reported unequal access to the new agents (thalidomide, bortezomib, and lenalidomide) in both public and private systems. Conversely, cheaper old drugs, represented by melphalan, were not available commercially in 44% (n = 7) of nations. Thus, first-line MM treatments for old and young patients in public practice were triplets with thalidomide-alkylating agent-steroid, whereas in private practice, treatments involved bortezomib-alkylating agent-steroid. An alarming rate of 30% of the nations reported suboptimal regimens (eg, VAD [vincristine, adriamycin, and dexamethasone]) or the impossibility of transplantation. CONCLUSION: Our data indicates that bortezomib and transplant are still an unmet medical necessity in public systems. In the complex puzzle of myeloma drug access in LATAMC, important issues, such as the adjustment of disparities between health systems, the incorporation of new drugs with an economic cost-effectiveness view, and the re-establishment of essential old drugs, can be a platform to the future.

Asunto(s)

RESUMEN

The number of allogeneic hematopoietic stem cell (HSC) transplants performed globally each year continues to increase. Advances in HLA typing, better supportive care, and administration of reduced-intensity conditioning regimens allow treatment of older patients with older sibling donors. Pretransplant donor assessment and testing are very important processes affecting the quality and safety of donation. For unrelated HSC donors detailed recommendations for health assessment have been published, allowing donation only if they are unrestrictedly healthy. Eligibility criteria for related donors are less strict and vary significantly between centers. In situations where a family donor does not meet the suitability criteria for unrelated donors, involved physicians often struggle with the decision whether the matched relative is suitable for donation or not. On behalf of the Worldwide Network for Blood and Marrow Transplantation Standing Committee on Donor Issues, we intended to develop a consensus document with recommendations for donor workup and final clearance of family donors who would not be able to serve as unrelated donors because of their age or pre-existing diseases. This article covers different topics intending to support decision-making, with the goal of minimizing medical risk to the donor and protection of the recipient from transmissible diseases.

Asunto(s)

RESUMEN

This study investigates the potential role of the recombinant c-mpl ligands (recombinant human thrombopoietin [rhTPO] and pegylated recombinant human megakaryocyte growth and development factor [PEG-rhMGDF]) on the recovery of platelet counts after TBI with and without allogeneic hematopoietic stem cell transplantation (HSCT) in an established canine model. Initially, 3 cohorts, each with 2 nonirradiated dogs, received increasing doses of rhTPO (5 microg/kg per day; 10 microg/kg per day; 20 microg/kg per day) for 7 days to determine the optimal dose. The dose of 10 microg/kg per day of rhTPO was selected for subsequent studies. Ten dogs then received either rhTPO or placebo for 28 days after 200 cGy TBI without HSCT. The rhTPO group had fewer days with platelet counts <20,000/microL (9.8 days versus 17.8 days, P < .05) and significantly increased granulocyte counts (n = 5) compared to the controls (n = 5). RhTPO-specific antibodies developed in 2 dogs, which caused a significant but transient decrease of the platelet counts. Retreatment of these sensitized dogs with rhTPO resulted in profound transient decreases in platelet counts. In the next study, 20 dogs received either PEG-rhMGDF or placebo for 21 days after 920 cGy TBI and allogeneic HSCT. The median time to platelet recovery (>20,000/microL) for the PEG-rhMGDF group (n = 10) was 14.0 days compared to 15.5 days for the control group (n = 10; log rank, P = .35). There were no significant differences in the total time to platelet counts <20,000/microL or in the time to recover neutrophil counts >500/microL. The effects of rhTPO on recovery of platelet and granulocyte counts after sublethal TBI were modest, and no effects of PEG-rhMGDF were observed on hematopoietic recovery after high-dose TBI and allogeneic HSCT. The significant effect that rhTPO-specific antibodies had on the platelet counts may limit the clinical role of recombinant c-mpl ligands unless sensitization can be prevented.

Asunto(s)

RESUMEN

Durante los últimos años se ha visto que el mejor conocimiento y manejo tanto de la fisiopatología de la cetoacidosis, como de la insulina-terapia y el manejo de los líquidos y electrolitos ha llevado a una disminución en las tasas de mortalidad, la cual fluctúa entre un (5-15 por ciento). Para resaltar tal importancia realizaremos una revisión bibliográfica de los aspectos más sobresalientes de esta patología, así como de un trabajo realizado en el Hospital Santo Tomás durante los años 1980 a 1984 en el cual se detallan los eventos ocurridos en la fisiopatología, su relación con la clínica y el por qué del tratamiento ha llevar a cabo

Asunto(s)