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1.
Biol Blood Marrow Transplant ; 26(4): 651-658, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-31917270

RESUMEN

Extracorporeal photopheresis (ECP) is an established treatment strategy in steroid-refractory graft-versus-host disease (GVHD). This study's main objective was to analyze the clinical response and impact of ECP therapy in steroid dose reduction. A retrospective observational series of 113 patients from 7 transplantation centers was analyzed. Sixty-five patients (58%) had acute GVHD (aGVHD), and 48 (42%) had chronic GVHD (cGVHD). All ECP procedures were performed with the off-line system. The median number of procedures until achievement of initial response was 3 for both patients with aGVHD and those with cGVHD. ECP was the second-line therapy in 48% of the aGVHD cases and in 50% of the cGVHD cases. 71% of the cases of aGVHD were grade III-IV, and 69% of the cases of cGVHD were severe. The overall response rate on day 28 was 53% (complete response [CR] rate, 45%) in the patients with aGVHD and 67% (CR, 23%) in those with cGVHD. Skin was the most frequently involved organ, with a response rate of 58% (CR, 49%) in the patients with aGVHD and 69% (CR 29%) in those with cGVHD. At the end of ECP treatment, 60% of patients treated for aGVHD who responded were able to stop steroid therapy, with a median dose reduction of 100%. Significant differences in overall survival were observed for patients responding to ECP with aGVHD (hazard ratio [HR], 4.3; P < .001) and with cGVHD (HR, 4.8; P = .003). Our data indicate that ECP is a valid therapeutic alternative in patients with steroid-refractory aGVHD and cGVHD, permitting significant steroid dosage reductions.


Asunto(s)
Enfermedad Injerto contra Huésped , Fotoféresis , Enfermedad Aguda , Enfermedad Crónica , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Humanos , Estudios Retrospectivos , Esteroides/uso terapéutico
2.
Haematologica ; 102(1): 85-93, 2017 01.
Artículo en Inglés | MEDLINE | ID: mdl-27540137

RESUMEN

The myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and myelofibrosis, are distinguished by their debilitating symptom profiles, life-threatening complications and profound impact on quality of life. The role gender plays in the symptomatology of myeloproliferative neoplasms remains under-investigated. In this study we evaluated how gender relates to patients' characteristics, disease complications and overall symptom expression. A total of 2,006 patients (polycythemia vera=711, essential thrombocythemia=830, myelofibrosis=460, unknown=5) were prospectively evaluated, with patients completing the Myeloproliferative Neoplasm-Symptom Assessment Form and Brief Fatigue Inventory Patient Reported Outcome tools. Information on the individual patients' characteristics, disease complications and laboratory data was collected. Consistent with known literature, most female patients were more likely to have essential thrombocythemia (48.6% versus 33.0%; P<0.001) and most male patients were more likely to have polycythemia vera (41.8% versus 30.3%; P<0.001). The rate of thrombocytopenia was higher among males than females (13.9% versus 8.2%; P<0.001) and males also had greater red-blood cell transfusion requirements (7.3% versus 4.9%; P=0.02) with shorter mean disease duration (6.4 versus 7.2 years, P=0.03). Despite there being no statistical differences in risk scores, receipt of most therapies or prior complications (hemorrhage, thrombosis), females had more severe and more frequent symptoms for most individual symptoms, along with overall total symptom score (22.8 versus 20.3; P<0.001). Females had particularly high scores for abdominal-related symptoms (abdominal pain/discomfort) and microvascular symptoms (headache, fatigue, insomnia, concentration difficulties, dizziness; all P<0.01). Despite complaining of more severe symptom burden, females had similar quality of life scores to those of males. The results of this study suggest that gender contributes to the heterogeneity of myeloproliferative neoplasms by influencing phenotypic profiles and symptom expression.


Asunto(s)
Trastornos Mieloproliferativos/epidemiología , Fenotipo , Calidad de Vida , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/mortalidad , Pronóstico , Factores Sexuales , Encuestas y Cuestionarios , Adulto Joven
3.
Blood ; 123(24): 3803-10, 2014 Jun 12.
Artículo en Inglés | MEDLINE | ID: mdl-24553173

RESUMEN

Symptom burden in myeloproliferative neoplasms (MPNs) is heterogeneous even among patients within the same MPN diagnosis. Using cluster analysis from prospectively gathered symptom burden data in 1470 international patients with essential thrombocythemia (ET), polycythemia vera (PV), or myelofibrosis (MF), we assessed for the presence of clusters and relationship to disease features and prognosis. In MF (4 clusters identified), clusters significantly differed by Dynamic International Prognostic Scoring System (DIPSS) risk (P < .001), leukopenia (P = .009), thrombocytopenia (P < .001), and spleen size (P = .02). Although an association existed between clusters and DIPSS risk, high symptom burden was noted in some low and intermediate-1-risk MF patients. In PV (5 clusters identified), total symptom score increased across clusters (P < .001), but clusters did not significantly differ by PV risk or the risk assessment variable of age. Among ET patients (5 clusters identified), clusters differed by gender (P = .04), anemia (P = .01), and prior hemorrhage (P = .047). Total symptom score increased across clusters (P < .001), but clusters did not significantly differ by International Prognostic Score for ET risk including the risk assessment variables. Significant symptom heterogeneity exists within each MPN subtype, sometimes independent of disease features or prognosis.


Asunto(s)
Neoplasias de la Médula Ósea/epidemiología , Trastornos Mieloproliferativos/epidemiología , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias de la Médula Ósea/complicaciones , Neoplasias de la Médula Ósea/diagnóstico , Análisis por Conglomerados , Femenino , Geografía , Humanos , Masculino , Persona de Mediana Edad , Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/diagnóstico , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiología , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/epidemiología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/epidemiología
5.
Transplantation ; 2024 Oct 22.
Artículo en Inglés | MEDLINE | ID: mdl-39437372

RESUMEN

Innovation is a hallmark of organ, tissue, and cell transplantation. The development of new treatments derived from these substances of human origin (SoHO) has rapidly evolved in recent years. Despite the great benefits that these innovative therapies could bring to patients, significant difficulties have arisen in making them equitably and widely accessible. Herein, we identify and address 4 challenges to promote innovation in this field in a collaborative, sustainable, and transparent manner and propose some concrete solutions applicable to SoHO-derived treatments, ranging from cell therapies to solid organ transplantation. Regulators, health policymakers, and government officials are recommended to incorporate specific elements into the regulatory frameworks of their respective jurisdictions, although regulatory convergence and equivalent quality and safety standards applicable to SoHO at a global level would be needed. An innovation-driven regulatory environment, respectful with the human origin and in accordance with the altruistic donation of SoHO, should be encouraged to improve the safety, effectiveness, accessibility, and affordability of SoHO and to promote collaboration between countries and between public and private sectors. This overview is the outcome of a working group focused on "Innovation in the donation and clinical application of SoHO" as part of the international Summit "Towards Global Convergence in Transplantation: Sufficiency, Transparency and Oversight" convened by the Organización Nacional de Trasplantes under the Spanish Presidency of the Council of the European Union in November 2023 and cosponsored by the Council of Europe, the World Health Organization, the Transplantation Society, and the European Society for Organ Transplantation.

6.
Dermatol Online J ; 19(3): 16, 2013 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-23552013

RESUMEN

Acral erythema is a frequent cutaneous reaction related to chemotherapy. A patient presented herein developed acral erythema related to cytosine arabinoside treatment and then graft versus host disease (GVHD). Subsequently, worsening of palmar erythema and pain occurred with intravenous cyclosporin infusions.


Asunto(s)
Ciclosporina/efectos adversos , Citarabina/uso terapéutico , Eritema/inducido químicamente , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Adulto , Antineoplásicos/uso terapéutico , Erupciones por Medicamentos/etiología , Eritema/terapia , Humanos , Infusiones Intravenosas , Masculino , Metotrexato/uso terapéutico
7.
Stem Cell Res Ther ; 13(1): 408, 2022 08 12.
Artículo en Inglés | MEDLINE | ID: mdl-35962457

RESUMEN

BACKGROUND: The increasing number of clinical trials for induced pluripotent stem cell (iPSC)-derived cell therapy products makes the production on clinical grade iPSC more and more relevant and necessary. Cord blood banks are an ideal source of young, HLA-typed and virus screened starting material to produce HLA-homozygous iPSC lines for wide immune-compatibility allogenic cell therapy approaches. The production of such clinical grade iPSC lines (haplolines) involves particular attention to all steps since donor informed consent, cell procurement and a GMP-compliant cell isolation process. METHODS: Homozygous cord blood units were identified and quality verified before recontacting donors for informed consent. CD34+ cells were purified from the mononuclear fraction isolated in a cell processor, by magnetic microbeads labelling and separation columns. RESULTS: We obtained a median recovery of 20.0% of the collected pre-freezing CD34+, with a final product median viability of 99.1% and median purity of 83.5% of the post-thawed purified CD34+ population. CONCLUSIONS: Here we describe our own experience, from unit selection and donor reconsenting, in generating a CD34+ cell product as a starting material to produce HLA-homozygous iPSC following a cost-effective and clinical grade-compliant procedure. These CD34+ cells are the basis for the Spanish bank of haplolines envisioned to serve as a source of cell products for clinical research and therapy.


Asunto(s)
Células Madre Pluripotentes Inducidas , Antígenos CD34/genética , Antígenos CD34/metabolismo , Bancos de Sangre , Sangre Fetal , Homocigoto , Células Madre Pluripotentes Inducidas/metabolismo
8.
Expert Rev Neurother ; 22(10): 897-903, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-36408604

RESUMEN

BACKGROUND: Therapeutic plasma exchange (TPE) was first used in neurology in the 1980s for myasthenia gravis (MG) and Guillain-Barré syndrome (GBS). Indications have since grown. Fear of complications with this treatment modality limit its use. RESEARCH DESIGN & METHODS: A study of patients undergoing TPE for neurological diseases (1981-2020) in a University Hospital in Madrid, Spain. Clinical indications, complications, procedure number, apheresis technique and replacement fluids were prospectively recorded and retrospectively analyzed. Historical trends were studied. RESULTS: 159 patients (48.69 ±18.15 years, 54.3% females) underwent TPE using central-venous catheter and replacement fluid albumin. We performed 1207 procedures over 189 cycles (6.4 ±3.8 procedures/cycle). Most patients underwent TPE for category I-II indications, mainly GBS and MG (77.7%). Complication rate was low (3.9% procedures), mostly hypotensive/vasovagal reactions (55.3%) and vascular access-related complications (38.3%). Most were mild-moderate (92.9%), permitting TPE completion, and somewhat more frequent during the first procedure (38.3%) and after periods of little TPE use. GBS patients were more prone to complications than MG patients (6.5% vs. 1.2%,p<0.001) mainly hypotensive/vasovagal reactions (3.7% vs. 1.0%,p=0.008). CONCLUSIONS: TPE is well-tolerated with low complication rate (<4% procedures), mainly hypotensive/vasovagal reactions. Patients with GBS seem more prone to them than MG patients. Acquaintance with this technique seems necessary.


Asunto(s)
Síndrome de Guillain-Barré , Intercambio Plasmático , Humanos , Síndrome de Guillain-Barré/terapia , Estudios Retrospectivos
9.
Am J Hematol ; 83(8): 673-5, 2008 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-18528824

RESUMEN

Reactivation of hepatitis B infection is an increasing problem for patients with lymphoma, even in resolved infections, who were treated with rituximab-based regimens. Our cases point out the need of prolonged prophylaxis in HBsAg-negative patients due to the high risk of developing fatal reactivation.


Asunto(s)
Anticuerpos Monoclonales/efectos adversos , Hepatitis B/inducido químicamente , Activación Viral/efectos de los fármacos , Anciano , Anticuerpos Monoclonales de Origen Murino , Femenino , Guanina/análogos & derivados , Guanina/uso terapéutico , Hepatitis B/tratamiento farmacológico , Humanos , Linfoma de Células B/complicaciones , Linfoma de Células B/tratamiento farmacológico , Persona de Mediana Edad , Rituximab , Resultado del Tratamiento
10.
Leuk Res ; 63: 34-40, 2017 12.
Artículo en Inglés | MEDLINE | ID: mdl-29096334

RESUMEN

Myelofibrosis is a myeloproliferative neoplasm associated with progressive cytopenias and high symptom burden. MF patients with thrombocytopenia have poor prognosis but the presence of thrombocytopenia frequently precludes the use of JAK2 inhibitors. In this study, we assessed quality of life and symptom burden in 418 MF patients with (n=89) and without (n=329) thrombocytopenia using prospective data from the MPN-QOL study group database, including the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) and Total Symptom Score (MPN10). Thrombocytopenia, defined as platelet count <100×109/L (moderate 51-100×109/L; severe ≤50×109/L), was associated with anemia (76% vs. 45%, p<0.001), leukopenia (29% vs. 11%, p<0.001), and need for red blood cell transfusion (35% vs. 19%, p=0.002). Thrombocytopenic patients had more fatigue, early satiety, inactivity, dizziness, sad mood, cough, night sweats, itching, fever, and weight loss; total symptom scores were also higher (33 vs. 24, p<0.001). Patients with severe thrombocytopenia were more likely to have anemia (86% vs. 67%, p=0.04), leukopenia (40% vs. 20%, p=0.04), and transfusion requirements (51% vs. 20%, p=0.002) but few differences in symptoms when compared to patients with moderate thrombocytopenia. These results suggest that MF patients with thrombocytopenia experience greater symptomatic burden than MF patients without thrombocytopenia and may benefit from additional therapies.


Asunto(s)
Evaluación de Necesidades , Mielofibrosis Primaria/diagnóstico , Índice de Severidad de la Enfermedad , Evaluación de Síntomas , Trombocitopenia/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Mielofibrosis Primaria/epidemiología , Pronóstico , Estudios Prospectivos , Calidad de Vida , Encuestas y Cuestionarios , Trombocitopenia/epidemiología
11.
J Nucl Med ; 47(10): 1643-8, 2006 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-17015900

RESUMEN

UNLABELLED: PET/CT combines functional and morphologic data and increases diagnostic accuracy in a variety of malignancies. This study prospectively compares the agreement between contrast-enhanced full-dose PET/CT and unenhanced low-dose PET/CT in lesion detection and initial staging of Hodgkin's disease and non-Hodgkin's lymphoma. METHODS: Forty-seven biopsy-proven lymphoma patients underwent a 18F-FDG PET/CT study that included unenhanced low-dose CT and enhanced full-dose CT for initial staging. Patients who had undergone previous diagnostic CT for initial staging were excluded. For every patient, each modality of PET/CT images was evaluated by either of 2 pairs of readers, with each pair comprising 1 experienced radiologist and 1 experienced nuclear physician. While evaluating one of the 2 types of PET/CT, the readers were unaware of the results of the other type. Lesion detection, number of sites affected in each anatomic region, and disease stage were assessed. Agreement between techniques was determined by the kappa-statistic, and discordances were studied by the McNemar test. Clinical, analytic, histopathologic, diagnostic CT, and PET data; data from other imaging techniques; and follow-up data constituted the reference standard. RESULTS: For region-based analysis, no significant differences were found between unenhanced low-dose PET/CT and contrast-enhanced full-dose PET/CT, although full-dose PET/CT showed fewer indeterminate findings and a higher number of extranodal sites affected than did low-dose PET/CT. Agreement between the 2 types of PET/CT was almost perfect for disease stage (kappa = 0.92; P < 0.001). CONCLUSION: Our study showed a good correlation between unenhanced low-dose PET/CT and contrast-enhanced full-dose PET/CT for lymph node and extranodal disease in lymphomas, suggesting that unenhanced low-dose PET/CT might suffice in most patients as the only imaging technique for the initial staging of lymphomas, reserving diagnostic CT for selected cases.


Asunto(s)
Enfermedad de Hodgkin/diagnóstico , Linfoma no Hodgkin/diagnóstico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Medios de Contraste , Femenino , Fluorodesoxiglucosa F18 , Enfermedad de Hodgkin/diagnóstico por imagen , Humanos , Linfoma no Hodgkin/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Tomografía de Emisión de Positrones , Estudios Prospectivos , Dosis de Radiación , Radiografía Abdominal , Esclerosis , Tomografía Computarizada por Rayos X
12.
Curr Probl Diagn Radiol ; 35(4): 151-63, 2006.
Artículo en Inglés | MEDLINE | ID: mdl-16814002

RESUMEN

The incidence of Hodgkin's disease (HD) and Non-Hodgkin's lymphoma (NHL) is around 8% of all malignancies. Fortunately, HD and NHL are among the few malignancies that are potentially curable with current existing treatment modalities, even in advanced or recurrent disease. Accurate staging, early therapy monitoring, and posttreatment evaluation of lymphomas are important for optimum management of these patients. We reviewed the imaging findings of patients with histologically proved lymphoma who underwent staging positron emission tomography/computed tomography (PET/CT), early monitoring therapy PET/CT (after 3 cycles of chemotherapy), and posttreatment PET/CT. PET/CT imaging findings are shown. Utility of PET/CT in recognizing false-positive and false-negative cases of CT and PET alone is addressed. Pitfalls and diagnostic difficulties are analyzed. PET/CT is a new imaging technology that improves the evaluation of lymphoma. This review will help the reader to better understand the imaging findings and applications of PET/CT in the management of lymphoma.


Asunto(s)
Enfermedad de Hodgkin/diagnóstico por imagen , Linfoma no Hodgkin/diagnóstico por imagen , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos X , Artefactos , Enfermedad de Hodgkin/terapia , Humanos , Linfoma no Hodgkin/terapia , Sensibilidad y Especificidad
13.
J Clin Oncol ; 34(2): 151-9, 2016 Jan 10.
Artículo en Inglés | MEDLINE | ID: mdl-26598745

RESUMEN

PURPOSE: Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) associated with disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden. PATIENTS AND METHODS: Through prospective evaluation of 1,334 patients with PV who had characterized symptom burden, we assessed patient demographics, laboratory data, and the presence of splenomegaly by disease feature (ie, known HU use, known phlebotomy requirements, splenomegaly). RESULTS: The presence of each feature in itself is associated with a moderately high symptom burden (MPN symptom assessment form [SAF] total symptom score [TSS] range, 27.7 to 29.2) that persists independent of PV risk category. In addition, symptoms incrementally increase in severity with the addition of other features. Patients with PV who had all three features (PV-HUPS) faced the highest total score (MPN-SAF TSS, 32.5) but had similar individual symptom scores to patients with known HU use (PV-HU), known phlebotomy (PV-P), and splenomegaly (PV-S). CONCLUSION: The results of this study suggest that patients with PV who have any one of the features in question (known HU use, known phlebotomy, or splenomegaly) have significant PV-associated symptoms. Furthermore, it demonstrates that many PV symptoms remain severe independent of the number of features present.


Asunto(s)
Antineoplásicos/efectos adversos , Hidroxiurea/efectos adversos , Flebotomía , Policitemia Vera/complicaciones , Policitemia Vera/tratamiento farmacológico , Esplenomegalia/etiología , Adulto , Anciano , Anciano de 80 o más Años , Antineoplásicos/administración & dosificación , Fatiga/etiología , Femenino , Fiebre/etiología , Humanos , Hidroxiurea/administración & dosificación , Quinasas Janus/antagonistas & inhibidores , Quinasas Janus/genética , Masculino , Persona de Mediana Edad , Dolor/etiología , Palpación , Pronóstico , Estudios Prospectivos , Prurito/etiología , Índice de Severidad de la Enfermedad , Sudoración , Pérdida de Peso
14.
Med Clin (Barc) ; 139(9): 373-8, 2012 Oct 13.
Artículo en Español | MEDLINE | ID: mdl-22743278

RESUMEN

BACKGROUND AND OBJECTIVES: Our study has investigated the presence of the mutation V617F in the JAK2 gene in patients diagnosed with chronic myeloproliferative neoplasms (MPNs). Furthermore, we determined if JAK2 (V617F) allelic burden associates with a specific clinical phenotype and if its quantification can be used as a marker to predict outcome and complications in patients with MPNs. PATIENTS AND METHODS: A retrospective observational study was conducted from 1987 to 2011 in the Haematology Department of La Paz University Hospital. The allelic burden was measured in 114 patients diagnosed with MPNs: 39 polycythemia vera (PV) patients, 71 essential thrombocythaemia patients and 6 primary myelofibrosis patients. The quantitative real-time polymerase chain reaction (qRT-PCR) technology was used to determinate the percentage of mutated alleles in peripheral blood neutrophils. Patients were divided in 2 groups: heterozygous if the result was≤50% of the tested cells, and homozygous if it was positive in>50% of the cells. RESULTS: Sixty-nine patients were positive for the JAK2 mutation and 45 were negative. Among those positive, the mutation was associated with arterial thrombosis. In addition, we demonstrate in the homozygous group that the V617F mutation is associated to PV, advanced age, leukocytosis, marked haematopoiesis and splenomegaly. CONCLUSIONS: The presence of V617F mutation is associated with a higher incidence of thrombosis, leukocytosis and splenomegaly. The identification of mutation on the JAK2 gene could help in a better definition of evolution and prognostic stratification of the myeloproliferative disorders.


Asunto(s)
Heterocigoto , Homocigoto , Janus Quinasa 2/genética , Trastornos Mieloproliferativos/genética , Fenotipo , Mutación Puntual , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Marcadores Genéticos , Humanos , Modelos Lineales , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/diagnóstico , Policitemia Vera/complicaciones , Policitemia Vera/diagnóstico , Policitemia Vera/genética , Mielofibrosis Primaria/complicaciones , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/genética , Pronóstico , Reacción en Cadena en Tiempo Real de la Polimerasa , Estudios Retrospectivos , Trombocitemia Esencial/complicaciones , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/genética
15.
J Clin Oncol ; 30(33): 4098-103, 2012 Nov 20.
Artículo en Inglés | MEDLINE | ID: mdl-23071245

RESUMEN

PURPOSE: Myeloproliferative neoplasm (MPN) symptoms are troublesome to patients, and alleviation of this burden represents a paramount treatment objective in the development of MPN-directed therapies. We aimed to assess the utility of an abbreviated symptom score for the most pertinent and representative MPN symptoms for subsequent serial use in assessing response to therapy. PATIENTS AND METHODS: The Myeloproliferative Neoplasm Symptom Assessment Form total symptom score (MPN-SAF TSS) was calculated as the mean score for 10 items from two previously validated scoring systems. Questions focus on fatigue, concentration, early satiety, inactivity, night sweats, itching, bone pain, abdominal discomfort, weight loss, and fevers. RESULTS: MPN-SAF TSS was calculable for 1,408 of 1,433 patients with MPNs who had a mean score of 21.2 (standard deviation [SD], 16.3). MPN-SAF TSS results significantly differed among MPN disease subtypes (P<.001), with a mean of 18.7 (SD, 15.3), 21.8 (SD, 16.3), and 25.3 (SD, 17.2) for patients with essential thrombocythemia, polycythemia vera, and myelofibrosis, respectively. The MPN-SAF TSS strongly correlated with overall quality of life (QOL; r=0.59; P<.001) and European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire C30 (EORTC QLQ-C30) functional scales (all P<.001 and absolute r≥0.50 except social functioning r=0.48). No significant trends were present when comparing therapy subgroups. The MPN-SAF TSS had excellent internal consistency (Cronbach's α=.83). Factor analysis identified a single underlying construct, indicating that the MPN-SAF TSS is an appropriate, unified scoring method. CONCLUSION: The MPN-SAF TSS is a concise, valid, and accurate assessment of MPN symptom burden with demonstrated clinical utility in the largest prospective MPN symptom study to date. This new prospective scoring method may be used to assess MPN symptom burden in both clinical practice and trial settings.


Asunto(s)
Trastornos Mieloproliferativos/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastornos Mieloproliferativos/patología , Estudios Prospectivos , Calidad de Vida , Índice de Severidad de la Enfermedad , Perfil de Impacto de Enfermedad , Encuestas y Cuestionarios , Adulto Joven
16.
Med Clin (Barc) ; 137(9): 383-9, 2011 Oct 08.
Artículo en Español | MEDLINE | ID: mdl-21703647

RESUMEN

BACKGROUND AND OBJECTIVE: To prospectively analyze the diagnostic accuracy of PET/CT in non-Hodgkin's lymphoma (NHL) and to evaluate the most appropriate study protocol of this technique. PATIENTS AND METHOD: Seventy-six biopsy proven NHL patients were enrolled in this prospective study for 3 years. Patients initially underwent a low-dose CT without intravenous contrast, then a PET emission scan and finally a full-dose CT with intravenous contrast. For every patient, two modalities of PET/CT images were reconstructed: a low-dose unenhanced PET/CT and a full-dose enhanced PET/CT. Each modality was evaluated by either of two pairs of readers, different for each modality. Enhanced CT and PET images were evaluated by an independent radiologist and nuclear medicine physician respectively. RESULTS: Agreement between reference standard and techniques was as follows: 52.2% of patients with enhanced CT (κ=0.458), 46% with PET (κ=0.335), 75% with low-dose unenhanced PET/CT (κ=0.664) and 76.8% with full-dose enhanced PET/CT (κ=0.679), with p<0.001. Although all techniques underestimated the stage in comparison to gold standard, the lowest percentage was for full-dose enhanced PET/CT (20.3%). CONCLUSIONS: PET/CT improved staging accuracy of NHL, being full-dose enhanced PET/CT the most accurate technique in our study.


Asunto(s)
Linfoma no Hodgkin/patología , Imagen Multimodal , Estadificación de Neoplasias/métodos , Adolescente , Adulto , Anciano , Biomarcadores de Tumor/sangre , Examen de la Médula Ósea , Medios de Contraste , Estudios Transversales , Femenino , Radioisótopos de Flúor , Fluorodesoxiglucosa F18 , Humanos , Linfoma no Hodgkin/sangre , Linfoma no Hodgkin/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias/normas , Medicina Nuclear , Variaciones Dependientes del Observador , Examen Físico , Tomografía de Emisión de Positrones , Estudios Prospectivos , Radiología , Radiofármacos , Estándares de Referencia , Reproducibilidad de los Resultados , Tomografía Computarizada por Rayos X , Adulto Joven
18.
Br J Haematol ; 135(3): 293-302, 2006 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-17032175

RESUMEN

An accurate initial staging of patients with non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) is critical for the selection of an appropriate treatment. Computed tomography (CT) remains the standard imaging technique, although it is based on anatomic criteria. Positron emission tomography (PET) with 2-deoxy-2-[fluorine-18]fluoro-d-glucose (FDG) provides useful functional information but requires anatomical correlation to localise lesions accurately. We have prospectively compared the accuracy of combined PET/CT with that of CT and PET alone at initial staging in lymphoma patients. Forty-seven newly diagnosed patients were evaluated. PET/CT was superior compared with CT and PET in nodal evaluation and detection of extranodal disease. Using a staging algorithm with PET/CT resulted in the disease stage being increased in 11 of 47 patients (10 NHL and 1 HL) (McNemar test P = 0.012). Therefore, a different treatment strategy based on PET/CT findings was suggested for seven patients (14.8%). PET/CT markedly improves accuracy in the diagnostic work-up of patients with lymphoma.


Asunto(s)
Linfoma/diagnóstico por imagen , Tomografía de Emisión de Positrones/métodos , Tomografía Computarizada por Rayos X/métodos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Algoritmos , Médula Ósea/patología , Femenino , Fluorodesoxiglucosa F18 , Enfermedad de Hodgkin/diagnóstico por imagen , Enfermedad de Hodgkin/tratamiento farmacológico , Humanos , Linfoma/tratamiento farmacológico , Linfoma no Hodgkin/diagnóstico por imagen , Linfoma no Hodgkin/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Estudios Prospectivos , Radiofármacos , Resultado del Tratamiento
19.
Med. clín (Ed. impr.) ; Med. clín (Ed. impr.);139(9): 373-378, oct. 2012. tab
Artículo en Español | IBECS (España) | ID: ibc-105481

RESUMEN

Fundamento y objetivo: La identificación de la mutación V617F del gen JAK2 en pacientes diagnosticados de neoplasias mieloproliferativas crónicas (NMPC) es fundamental en el cribado diagnóstico. Nuestro objetivo fue investigar la asociación entre la cuantificación de la mutación V617F (carga alélica JAK2V617F) y el fenotipo clínico al diagnóstico, y definir el papel de la carga alélica en la predicción de complicaciones. Pacientes y métodos: En el Servicio de Hematología del Hospital Universitario La Paz realizamos un estudio observacional retrospectivo (1987-2011) en 114 pacientes diagnosticados de NMPC y análisis de detección de la mutación V617F: 39 policitemias vera (PV), 71 trombocitemias esenciales y 6 mielofibrosis primarias. El porcentaje de alelos mutados fue evaluado en polimorfonucleares de sangre periférica mediante la técnica quantitative real time polymerase chain reaction (qRT-PCR, «reacción en cadena de la polimerasa cuantitativa en tiempo real»). En función de si la carga tumoral se encuentra entre 1-50% o es>50% los pacientes fueron diagnosticados de JAK2mut heterocigoto u homocigoto, respectivamente. Resultados: Se realizó el análisis cuantitativo por qRT-PCR en los 114 pacientes incluidos en el estudio, detectando la mutación V617F en 69 casos y siendo negativa para los 45 pacientes restantes. Encontramos que la presencia de mutación se asocia con la trombosis, y especialmente con la trombosis arterial. Además, y en la serie completa, la carga alélica homocigótica se asocia con diagnóstico de PV, edad avanzada, leucocitosis, mayor hematopoyesis y esplenomegalia. Conclusiones: La detección de la mutación V617F está asociada a una mayor incidencia de trombosis, leucocitosis y esplenomegalia. Su identificación nos permitiría una mejor estratificación pronóstica de los pacientes diagnosticados de NMPC (AU)


Background and objectives: Our study has investigated the presence of the mutation V617F in the JAK2 gene in patients diagnosed with chronic myeloproliferative neoplasms (MPNs). Furthermore, we determined if JAK2 (V617F) allelic burden associates with a specific clinical phenotype and if its quantification can be used as a marker to predict outcome and complications in patients with MPNs.Patients and methods: A retrospective observational study was conducted from 1987 to 2011 in the Haematology Department of La Paz University Hospital. The allelic burden was measured in 114 patients diagnosed with MPNs: 39 polycythemia vera (PV) patients, 71 essential thrombocythaemia patients and 6 primary myelofibrosis patients. The quantitative real-time polymerase chain reaction (qRT-PCR) technology was used to determinate the percentage of mutated alleles in peripheral blood neutrophils. Patients were divided in 2 groups: heterozygous if the result was≤50% of the tested cells, and homozygous if it was positive in>50% of the cells. Results: Sixty-nine patients were positive for the JAK2 mutation and 45 were negative. Among those positive, the mutation was associated with arterial thrombosis. In addition, we demonstrate in the homozygous group that the V617F mutation is associated to PV, advanced age, leukocytosis, marked haematopoiesis and splenomegaly. Conclusions: The presence of V617F mutation is associated with a higher incidence of thrombosis, leukocytosis and splenomegaly. The identification of mutation on the JAK2 gene could help in a better definition of evolution and prognostic stratification of the myeloproliferative disorders (AU)


Asunto(s)
Humanos , Trastornos Mieloproliferativos/genética , Neoplasias Hematológicas/genética , Cromosoma Filadelfia , Quinasas Janus/análisis , Mutación , Estudios Retrospectivos , Leucocitosis/genética , Esplenomegalia/etiología
20.
Med. clín (Ed. impr.) ; Med. clín (Ed. impr.);137(9): 383-389, oct. 2011.
Artículo en Español | IBECS (España) | ID: ibc-91899

RESUMEN

Fundamento y objetivo: Analizar prospectivamente la precisión de la tomografía por emisión de positrones/tomografía computarizada (PET/TC) frente al patrón oro en la estadificación inicial de los pacientes con linfoma no hodgkiniano (LNH) e identificar el protocolo técnico más adecuado.Pacientes y método: Estudio transversal prospectivo de 76 pacientes diagnosticados de LNH durante 3 años consecutivos en el Hospital Universitario La Paz de Madrid no tratados previamente. Todos los pacientes fueron estudiados con la técnica PET/TC: inicialmente se obtuvo una TC de dosis baja, después el escáner de emisión de la PET y posteriormente un estudio de TC de dosis alta con contraste intravenoso. Se reconstruyeron dos estudios de PET/TC para cada paciente: uno de PET/TC de dosis baja (PET/TC DB) y otro de PET/TC de dosis alta (PET/TC DA). Cada modalidad de PET/TC fue evaluada en consenso por un equipo de médico nuclear y radiólogo, diferente para cada una de las modalidades. Las imágenes de TC y de PET fueron interpretadas por separado por otro radiólogo y otro médico nuclear independientes.Resultados: La concordancia con el patrón de referencia fue en el 52,2% de los pacientes (κ=0,458) para la TC, 46% para la PET (κ=0,335), 75% para la PET/TC DB (κ=0,664) y 76,8% para la PET/TC DA (κ=0,679), con p<0,001. Todas las técnicas infravaloraron el estadio (p<0,05), aunque con la PET/TC DA en un menor porcentaje de pacientes (20,3%). Conclusión: La PET/TC es superior en la estadificación inicial de los pacientes con LNH, siendo la PET/TC DA la técnica más precisa de las que hemos estudiado (AU)


Background and objective: To prospectively analyze the diagnostic accuracy of PET/CT in non-Hodgkin's lymphoma (NHL) and to evaluate the most appropriate study protocol of this technique.Patients and method: Seventy-six biopsy proven NHL patients were enrolled in this prospective study for 3 years. Patients initially underwent a low-dose CT without intravenous contrast, then a PET emission scan and finally a full-dose CT with intravenous contrast. For every patient, two modalities of PET/CT images were reconstructed: a low-dose unenhanced PET/CT and a full-dose enhanced PET/CT. Each modality was evaluated by either of two pairs of readers, different for each modality. Enhanced CT and PET images were evaluated by an independent radiologist and nuclear medicine physician respectively.Results: Agreement between reference standard and techniques was as follows: 52.2% of patients with enhanced CT (κ=0.458), 46% with PET (κ=0.335), 75% with low-dose unenhanced PET/CT (κ=0.664) and 76.8% with full-dose enhanced PET/CT (κ=0.679), with p<0.001. Although all techniques underestimated the stage in comparison to gold standard, the lowest percentage was for full-dose enhanced PET/CT (20.3%).Conclusions: PET/CT improved staging accuracy of NHL, being full-dose enhanced PET/CT the most accurate technique in our study (AU)


Asunto(s)
Humanos , Tomografía de Emisión de Positrones/métodos , Linfoma no Hodgkin/patología , /métodos , Antineoplásicos/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico
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