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1.
Blood ; 135(4): 274-286, 2020 01 23.
Artículo en Inglés | MEDLINE | ID: mdl-31738823

RESUMEN

Pediatric large B-cell lymphomas (LBCLs) share morphological and phenotypic features with adult types but have better prognosis. The higher frequency of some subtypes such as LBCL with IRF4 rearrangement (LBCL-IRF4) in children suggests that some age-related biological differences may exist. To characterize the genetic and molecular heterogeneity of these tumors, we studied 31 diffuse LBCLs (DLBCLs), not otherwise specified (NOS); 20 LBCL-IRF4 cases; and 12 cases of high-grade B-cell lymphoma (HGBCL), NOS in patients ≤25 years using an integrated approach, including targeted gene sequencing, copy-number arrays, and gene expression profiling. Each subgroup displayed different molecular profiles. LBCL-IRF4 had frequent mutations in IRF4 and NF-κB pathway genes (CARD11, CD79B, and MYD88), losses of 17p13 and gains of chromosome 7, 11q12.3-q25, whereas DLBCL, NOS was predominantly of germinal center B-cell (GCB) subtype and carried gene mutations similar to the adult counterpart (eg, SOCS1 and KMT2D), gains of 2p16/REL, and losses of 19p13/CD70. A subset of HGBCL, NOS displayed recurrent alterations of Burkitt lymphoma-related genes such as MYC, ID3, and DDX3X and homozygous deletions of 9p21/CDKN2A, whereas other cases were genetically closer to GCB DLBCL. Factors related to unfavorable outcome were age >18 years; activated B-cell (ABC) DLBCL profile, HGBCL, NOS, high genetic complexity, 1q21-q44 gains, 2p16/REL gains/amplifications, 19p13/CD70 homozygous deletions, and TP53 and MYC mutations. In conclusion, these findings further unravel the molecular heterogeneity of pediatric and young adult LBCL, improve the classification of this group of tumors, and provide new parameters for risk stratification.


Asunto(s)
Factores Reguladores del Interferón/genética , Linfoma de Células B Grandes Difuso/genética , Mutación , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/patología , Masculino , Pronóstico , Transcriptoma , Adulto Joven
2.
Pediatr Dermatol ; 38(2): 516-517, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33486776

RESUMEN

The net-like superficial lymphatic malformation (LM) is a newly described entity with distinctive clinical, dermoscopic, and histologic characteristics. Clinical picture consists of red to purplish macules with a finely reticulated pattern of vascular structures. Dermoscopy shows arborizing telangiectatic vessels. Histology is characterized by a vascular proliferation composed of thin-walled vessels, located in the upper dermis, that stains positive with podoplanin (D2-40). We report a new case of LM with an additional clinical feature, hypopigmented areas.


Asunto(s)
Anomalías Linfáticas , Neoplasias Cutáneas , Telangiectasia , Dermoscopía , Humanos
8.
Ophthalmic Plast Reconstr Surg ; 30(5): e131-4, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24317101

RESUMEN

Natural killer/T-cell lymphoma (NKTCL) and its presentation with extranodal orbital involvement as a single lesion are extremely rare. The aim of this article was to describe the presentation, diagnosis, and systemic treatment of a primary orbital NKTCL. A 67-year-old Caucasian woman presented with left exophthalmos, pain, periorbital swelling, and limited extrinsic ocular motility. Orbital cellulitis was suspected, but finally orbital biopsy was performed due to no response to initial antibiotic and anti-inflammatory standard treatment. The pathologic diagnosis was NKTCL. Systemic evaluations were negative. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy was initiated, but after 2 cycles of treatment, tumoral progression was observed. SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) rescue chemotherapy was then administered. Lymphoma progression was inevitable. She died 10 months later. Although more nasal NKTCL cases have been described, the nonnasal primary orbital NKTCL is an uncommon neoplasm with high mortality rate, despite the recent use of more potent chemotherapy regimens.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Linfoma Extranodal de Células NK-T/tratamiento farmacológico , Linfoma/tratamiento farmacológico , Neoplasias Orbitales/tratamiento farmacológico , Anciano , Exoftalmia/diagnóstico , Dolor Ocular/diagnóstico , Resultado Fatal , Femenino , Humanos , Linfoma/diagnóstico por imagen , Linfoma/patología , Linfoma Extranodal de Células NK-T/diagnóstico por imagen , Linfoma Extranodal de Células NK-T/patología , Neoplasias Orbitales/diagnóstico por imagen , Neoplasias Orbitales/patología , Tomografía Computarizada por Rayos X
9.
Ital J Dermatol Venerol ; 159(4): 390-411, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39069838

RESUMEN

Lentigo maligna (LM), a form of melanoma in situ, and LM melanoma (LMM), its invasive counterpart, exhibit distinctive epidemiology, risk factors, and clinical features compared to other melanoma subtypes. Notably, LM occurs on chronically sun-damaged skin presenting as a slow-growing, ill-defined patch which makes it difficult to diagnose and to treat. Additionally, while LM generally presents a favourable prognosis, it can also lead to dermal invasion and behave similarly to other melanomas with the same Breslow thickness. Hence, surgery continues to be the cornerstone treatment. Wide excisions are often necessary, but challenges arise when these lesions manifest in cosmetically sensitive regions, limiting the feasibility and desirability of large excisions. Specialized approaches, including margin-controlled surgery and image-guided treatment with reflectance confocal microscopy, have been developed to address these issues. Other non-surgical treatments such as cryosurgery, imiquimod, radiotherapy, or photodynamic therapy, may also be used but commonly present with recurrent/persistent disease. Herein we comprehensively review the existing literature on the management of LM/LMM, and discus the potential new advances on managing this challenging skin cancer.


Asunto(s)
Peca Melanótica de Hutchinson , Neoplasias Cutáneas , Humanos , Peca Melanótica de Hutchinson/terapia , Peca Melanótica de Hutchinson/diagnóstico , Neoplasias Cutáneas/terapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Fotoquimioterapia , Imiquimod/uso terapéutico
10.
J Infect Dis ; 203(6): 810-3, 2011 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-21343147

RESUMEN

We studied the lymphoid tissue biopsies of 20 patients with chronic human immunodeficiency virus (HIV) infection by analyzing collagen deposition, CD4+ cell number, and gene expression of metalloproteinases (MMPs; MMP-2, MMP-9) and tissue inhibitors of MMPs (TIMPs; TIMP-1, TIMP-2). HIV-infected patients had significantly increased collagen deposition (P = .001), fewer CD4+ T cells (P = .05), and decreased MMP-9/TIMP-1 and MMP-2/TIMP-2 ratios (P = .01), compared with HIV-negative control patients. Moreover, we found a significant negative correlation between collagen deposition and the MMP-9/TIMP-1 ratio (ρ = -0.50; P = .047). To our knowledge, this is the first time that MMP/TIMP imbalance has been correlated with lymphoid tissue collagen deposition and incomplete immune recovery in HIV-infected patients, even after long-term antiretroviral treatment.


Asunto(s)
Colágeno/metabolismo , Infecciones por VIH/metabolismo , Metaloproteinasa 2 de la Matriz/metabolismo , Metaloproteinasa 9 de la Matriz/metabolismo , Inhibidor Tisular de Metaloproteinasa-1/metabolismo , Inhibidor Tisular de Metaloproteinasa-2/metabolismo , Adulto , Antirretrovirales/uso terapéutico , Linfocitos T CD4-Positivos , Femenino , Regulación Enzimológica de la Expresión Génica , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/inmunología , Humanos , Tejido Linfoide/metabolismo , Masculino , Metaloproteinasa 2 de la Matriz/genética , Metaloproteinasa 9 de la Matriz/genética , Persona de Mediana Edad , Tonsila Palatina/metabolismo , Tonsila Palatina/patología , Reacción en Cadena de la Polimerasa , Análisis de Regresión , Inhibidor Tisular de Metaloproteinasa-1/genética , Inhibidor Tisular de Metaloproteinasa-2/genética
11.
J Med Case Rep ; 15(1): 326, 2021 Jun 26.
Artículo en Inglés | MEDLINE | ID: mdl-34172079

RESUMEN

BACKGROUND: We describe a patient with blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement and the outcome of venetoclax use in this setting. CASE PRESENTATION: A 54-year-old Caucasian male was referred to the Haematology Unit with an enlarged inguinal lymph node which was diagnostic of a blastic plasmacytoid dendritic cell neoplasm. The staging revealed disseminated disease (skin, visceral, lymph nodes, and bone marrow). He received chemotherapy with an acute myeloid leukaemia-like regime. Afterwards, he underwent allogeneic haematopoietic stem cell transplantation, though it was not successful, showing a relapse 14 months later with hepatic and central nervous system dissemination. Intrathecal chemotherapy was administered, and venetoclax (anti-bcl2 agent) was started in an off-label indication based on most recent literature. The disease halted its course for 3 months. In the end, the patient's disease progressed and so he succumbed due to infectious complications. CONCLUSIONS: Venetoclax monotherapy seems not enough to control the disease progression under CNS involvement and other treatments should be investigated.


Asunto(s)
Células Dendríticas , Neoplasias Cutáneas , Compuestos Bicíclicos Heterocíclicos con Puentes , Sistema Nervioso Central , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Sulfonamidas
12.
Eur J Dermatol ; 30(4): 397-403, 2020 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-32815814

RESUMEN

BACKGROUND: Advanced-stage mycosis fungoides/Sézary syndrome (aMF/SS) has a dismal outcome. The only curative treatment is allogeneic stem cell transplantation (allo-SCT) but this is limited to selected candidates, thus palliative therapy is the most frequent strategy. OBJECTIVES: To describe the characteristics of aMF/SS in cases referred to haematology units for advanced/palliative therapy. MATERIALS AND METHODS: Data from 30 patients were collected from four centres, and descriptive statistics, frequencies and survival analyses were calculated. RESULTS: Eighty-eight per cent of patients received systemic therapy. The median number of therapies was three (range: 1-9). Bexarotene (21%), CHOP-like chemotherapy (10%) and methotrexate (9%) were the more common treatments. The overall survival at a median follow-up of 28 months (range: 8-65 months) for aMF/SS was 56.9%. Survival probability was more favourable for MF (p < 0.02). Nine patients received allo-SCT. Half of the patients (56%) relapsed after allo-SCT but could be rescued with immunosuppression tapering, donor lymphocyte infusions and additional therapy (80%). CONCLUSION: There is significant heterogeneity in aMF/SS treatments. Survival is more favourable for MF compared to SS. Current chemoimmunotherapies are insufficient to control disease, making allo-SCT the best therapeutic approach in selected patients.


Asunto(s)
Micosis Fungoide/terapia , Cuidados Paliativos , Síndrome de Sézary/terapia , Neoplasias Cutáneas/terapia , Adulto , Anciano , Anciano de 80 o más Años , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Bexaroteno/uso terapéutico , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Femenino , Trasplante de Células Madre Hematopoyéticas , Humanos , Inmunosupresores/uso terapéutico , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Micosis Fungoide/patología , Estadificación de Neoplasias , Prednisona/uso terapéutico , Derivación y Consulta , Estudios Retrospectivos , Síndrome de Sézary/patología , Neoplasias Cutáneas/patología , España , Análisis de Supervivencia , Trasplante Homólogo , Vincristina/uso terapéutico
13.
Clin Case Rep ; 8(7): 1269-1273, 2020 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-32695373

RESUMEN

Only proven pathogenic mutations associated with myeloid neoplasms are key to establish the clonal nature of the bone marrow fibrosis. In cases with genetic variants of uncertain meaning, the clinical picture may be required to rule out secondary causes.

14.
Hematol Rep ; 11(1): 7841, 2019 Feb 19.
Artículo en Inglés | MEDLINE | ID: mdl-30915203

RESUMEN

Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of Tcell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type. We report the case of a cutaneous TCR-silent type lymphoma associated to a clonal plasma cell proliferation with an ominous outcome that led to a lot of discussion in its classification. Due to the aggressiveness of the disease and the scant evidence about therapy in this strange entity the outcome was fatal. We report a unique case of a TCR-silent cutaneous TCL with an exceptional histopathology, prolonged clinical evolution and a subsequent plasma cell clonal expansion.

15.
Am J Surg Pathol ; 41(7): 877-886, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28288039

RESUMEN

MYC translocation is a defining feature of Burkitt lymphoma (BL), and the new category of high-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 translocations, and occurs in 6% to 15% of diffuse large B-cell lymphomas (DLBCLs). The low incidence of MYC translocations in DLBCL makes the genetic study of all these lymphomas cumbersome. Strategies based on an initial immunophenotypic screening to select cases with a high probability of carrying the translocation may be useful. LMO2 is a germinal center marker expressed in most lymphomas originated in these cells. Mining gene expression profiling studies, we observed LMO2 downregulation in BL and large B-cell lymphoma (LBCL) with MYC translocations, and postulated that LMO2 protein expression could assist to identify such cases. We analyzed LMO2 protein expression in 46 BLs and 284 LBCL. LMO2 was expressed in 1/46 (2%) BL cases, 146/268 (54.5%) DLBCL cases, and 2/16 (12.5%) high-grade B-cell lymphoma cases with MYC and BCL2 and/or BCL6 translocations. All BLs carried MYC translocation (P<0.001), whereas LMO2 was only positive in 6/42 (14%) LBCL with MYC translocation (P<0.001). The relationship between LMO2 negativity and MYC translocation was further analyzed in different subsets of tumors according to CD10 expression and cell of origin. Lack of LMO2 expression was associated with the detection of MYC translocations with high sensitivity (87%), specificity (87%), positive predictive value and negative predictive value (74% and 94%, respectively), and accuracy (87%) in CD10 LBCL. Comparing LMO2 and MYC protein expression, all statistic measures of performance of LMO2 surpassed MYC in CD10 LBCL. These findings suggest that LMO2 loss may be a good predictor for the presence of MYC translocation in CD10 LBCL.


Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/metabolismo , Biomarcadores de Tumor/metabolismo , Linfoma de Burkitt/metabolismo , Genes myc , Proteínas con Dominio LIM/metabolismo , Linfoma de Células B Grandes Difuso/metabolismo , Proteínas Proto-Oncogénicas/metabolismo , Translocación Genética , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Linfoma de Burkitt/genética , Linfoma de Burkitt/patología , Niño , Preescolar , Femenino , Regulación Neoplásica de la Expresión Génica , Humanos , Inmunohistoquímica , Linfoma de Células B Grandes Difuso/genética , Linfoma de Células B Grandes Difuso/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven
16.
Leuk Lymphoma ; 57(5): 1060-6, 2016 May.
Artículo en Inglés | MEDLINE | ID: mdl-27096891

RESUMEN

Mycosis fungoides and Sézary syndrome (MF/SS) are the most common forms of primary cutaneous T cell lymphomas. We analyzed the applicability of the cutaneous lymphoma international prognostic index (CLIPi) in MF/SS. We introduced the total body-surface area affected (TBSA) and the type of skin lesions at diagnosis as prognostic variables. The overall survival (OS) at median time of follow up (96 months) was 75.6% (CI 95%, 62.0-98.5%). In the univariate analysis, age>60 years, advanced disease, type of skin lesions and TBSA>50 showed poorer OS (p<0.05). In the multivariate analysis there was a significant increased relative risk of death in those patients>60 years, with advanced disease and TBSA>50% (p<0.05). TBSA identified a group of poor prognosis patients with advanced MF/SS that may benefit from novel systemic therapies.


Asunto(s)
Superficie Corporal , Micosis Fungoide/diagnóstico , Síndrome de Sézary/diagnóstico , Neoplasias Cutáneas/diagnóstico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor , Niño , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Micosis Fungoide/mortalidad , Estadificación de Neoplasias , Pronóstico , Modelos de Riesgos Proporcionales , Síndrome de Sézary/mortalidad , Neoplasias Cutáneas/mortalidad , Tomografía Computarizada por Rayos X , Adulto Joven
19.
Arch Bronconeumol ; 49(5): 210-2, 2013 May.
Artículo en Inglés, Español | MEDLINE | ID: mdl-22981515

RESUMEN

The bronchial involvement of diffuse large B-cell lymphoma (DLBCL) is an exceptional finding. Histological diagnosis is done with lung tissue samples. In these cases, the need for immunohistochemistry studies in order to establish the diagnosis requires obtaining tissue samples of adequate size and quality. Sometimes, endoscopic explorations may be repeated to obtain further biopsies. We present the first documented case of recurrent endobronchial DLBCL that was diagnosed from a bronchial biopsy taken with a cryoprobe.


Asunto(s)
Biopsia/métodos , Bronquios/patología , Criocirugía/métodos , Linfoma de Células B Grandes Difuso/diagnóstico , Anciano , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Cisplatino/administración & dosificación , Terapia Combinada , Ciclofosfamida/administración & dosificación , Citarabina/administración & dosificación , Diagnóstico Diferencial , Doxorrubicina/administración & dosificación , Etopósido/administración & dosificación , Humanos , Neoplasias Pulmonares/diagnóstico , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/patología , Linfoma de Células B Grandes Difuso/cirugía , Masculino , Metilprednisolona/administración & dosificación , Prednisona/administración & dosificación , Recurrencia , Inducción de Remisión , Rituximab , Bazo/patología , Esplenectomía , Esplenomegalia/etiología , Esplenomegalia/cirugía , Vincristina/administración & dosificación
20.
AIDS ; 24(13): 2029-39, 2010 Aug 24.
Artículo en Inglés | MEDLINE | ID: mdl-20588162

RESUMEN

OBJECTIVE: The factors associated with fibrosis in lymphoid tissue in long-term treated HIV-infected patients and their correlation with immune reconstitution were assessed. METHODS: Tonsillar biopsies were performed in seven antiretroviral-naive patients and 29 successfully treated patients (median time on treatment, 61 months). Twenty patients received protease inhibitors-sparing regimens and nine protease inhibitor-containing regimens. Five tonsillar resections of HIV-negative individuals were used as controls. Lymphoid tissue architecture, collagen deposition (fibrosis) and the mean interfollicular CD4(+) cell count per mum were assessed. RESULTS: Naive and long-term treated HIV-infected patients had a higher proportion of fibrosis than did HIV-uninfected persons (P < 0.001). Patients with greater collagen deposition showed lower levels of CD4 cells in lymphoid tissue (P = 0.03) and smaller increase in peripheral CD4(+) T cells (r = -0.40, P = 0.05). The factors independently associated with fibrosis in lymphoid tissue were age (P < 0.0001), treated patients with detectable lymphoid tissue viral load when compared with patients with undetectable lymphoid tissue viral load (median 5 vs. 12%, respectively, P = 0.017) and patients receiving a protease inhibitor-sparing vs. a protease inhibitor-containing regimen (median 8 vs. 2.5%, respectively, P = 0.04). CONCLUSION: Fibrosis in lymphoid tissue was associated with a poor reconstitution of CD4(+) T cells and long-term antiretroviral therapy did not reverse this abnormality. HIV infection, older age, a detectable level of lymphoid tissue viral load in treated patients and protease inhibitor-sparing regimens seem to favour fibrosis in lymphoid tissue.


Asunto(s)
Envejecimiento/metabolismo , Colágeno/metabolismo , Infecciones por VIH/patología , VIH-1/aislamiento & purificación , Tonsila Palatina/patología , Adulto , Envejecimiento/inmunología , Recuento de Linfocito CD4 , Colágeno/inmunología , Femenino , Fibrosis , Infecciones por VIH/inmunología , Infecciones por VIH/virología , Humanos , Masculino , Persona de Mediana Edad , Tonsila Palatina/virología , Factores de Riesgo , Carga Viral
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