Assuntos
Cromossomos Humanos Par 16/genética , Leucemia Mieloide Aguda/genética , Animais , Linhagem Celular Tumoral , Inversão Cromossômica/genética , Subunidade alfa 2 de Fator de Ligação ao Core/genética , Fatores de Ligação ao Core/genética , Proteínas de Ligação a DNA/genética , Humanos , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Fatores de Transcrição/genéticaRESUMO
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 74â 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.
Assuntos
Cadeias Leves de Imunoglobulina/metabolismo , Pulmão/patologia , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma não Hodgkin/genética , Síndrome de Sjogren/genética , Neoplasias Gástricas/genética , Adulto , Idoso , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/diagnóstico por imagem , Linfoma não Hodgkin/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Paraproteinemias , Síndrome de Sjogren/diagnóstico por imagem , Neoplasias Gástricas/diagnóstico por imagem , Espectrometria de Massas em TandemRESUMO
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.
