RESUMEN
The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of patients with marginal zone lymphoma (MZL).
Asunto(s)
Humanos , Linfoma de Células B de la Zona Marginal/diagnóstico , Biopsia , Linfoma de Células B de la Zona Marginal/clasificación , Linfoma Extranodal de Células NK-TAsunto(s)
Agammaglobulinemia Tirosina Quinasa/antagonistas & inhibidores , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/etiología , Sistema Cardiovascular/efectos de los fármacos , Inhibidores de Proteínas Quinasas/efectos adversos , Animales , Enfermedades Cardiovasculares/terapia , Toma de Decisiones Clínicas , Diagnóstico Diferencial , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Humanos , Inhibidores de Proteínas Quinasas/administración & dosificación , Inhibidores de Proteínas Quinasas/uso terapéutico , Factores de RiesgoRESUMEN
Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes (POEMS) syndrome is a rare disease, and only in a minority of cases, causes an impairment of kidney function. Here, we describe a case of a 55-year-old man with a history of POEMS syndrome who presented with acute kidney injury following a routine blood test. On further investigation, a relapse in POEMS syndrome was diagnosed, uniquely isolated to renal involvement.
Asunto(s)
Lesión Renal Aguda/etiología , Síndrome POEMS/complicaciones , Lesión Renal Aguda/fisiopatología , Humanos , Riñón/fisiopatología , Masculino , Persona de Mediana Edad , Síndrome POEMS/fisiopatología , RecurrenciaAsunto(s)
Antineoplásicos/uso terapéutico , Leucemia Linfocítica Crónica de Células B/tratamiento farmacológico , Antineoplásicos/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Contraindicaciones de los Medicamentos , Fragilidad , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Leucemia Linfocítica Crónica de Células B/genética , Mutación , Cuidados Paliativos/métodos , Ensayos Clínicos Controlados Aleatorios como Asunto , Recurrencia , Proteína p53 Supresora de Tumor/genéticaRESUMEN
The British Committee for Standards in Haematology first produced guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant in 2000. This revision updates those guidelines and covers the areas of diagnosis, treatment and assessment of response to therapy.
Asunto(s)
Leucemia de Células Pilosas/diagnóstico , Leucemia de Células Pilosas/terapia , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
The t(14;19)(q32;q13), involving the BCL3 locus at chromosome 19q13 and the immunoglobulin heavy chain gene at 14q32, is a rare recurrent cytogenetic abnormality identified in B-cell neoplasms, most of which have been classified as chronic lymphocytic leukaemia (CLL) in the literature. We describe the clinicopathological, immunophenotypic and cytogenetic findings in seven patients with B-cell neoplasms associated with t(14;19)(q32;q13). There were five men and two women, with a median age of 48 years (range 33-68). All had absolute lymphocytosis, six had lymphadenopathy, and one had splenomegaly. Lymphocytes in blood and bone marrow aspirate smears were predominantly small and cytologically atypical. Flow cytometric immunophenotyping showed an atypical immunophenotype with low CLL scores. The growth pattern in bone marrow biopsy specimens was interstitial to diffuse; immunohistochemical stains were positive for bcl3 and negative for cyclin D1. Lymph node biopsy specimens of two patients revealed total architectural effacement by neoplasm with proliferation centres. In addition to t(14;19), cytogenetic studies demonstrated trisomy 12 in five patients. These results suggest that B-cell neoplasms with the t(14;19)(q32;q13) present frequently as leukaemia composed of small B-lymphocytes and share many features with CLL. However, these neoplasms also differ from CLL cytologically and in their immunophenotype.
Asunto(s)
Cromosomas Humanos Par 14 , Cromosomas Humanos Par 19 , Leucemia de Células B/genética , Translocación Genética , Adulto , Proteínas del Linfoma 3 de Células B , Bandeo Cromosómico , Femenino , Citometría de Flujo , Humanos , Cadenas Pesadas de Inmunoglobulina/genética , Inmunofenotipificación , Hibridación Fluorescente in Situ , Cariotipificación , Leucemia de Células B/clasificación , Leucemia Linfocítica Crónica de Células B/clasificación , Masculino , Persona de Mediana Edad , Proteínas Proto-Oncogénicas/genética , Factores de Transcripción/genéticaRESUMEN
We retrospectively reviewed time to response and incidence of delayed responses in 13 patients with lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia (LPL/WM) treated with fludarabine with or without cyclophosphamide. During follow-up post-treatment, seven delayed responses (54%) were observed, improving the initial overall response rate of 61% to a final response rate of 77%.
Asunto(s)
Antineoplásicos/farmacología , Leucemia Linfocítica Crónica de Células B/tratamiento farmacológico , Vidarabina/análogos & derivados , Macroglobulinemia de Waldenström/tratamiento farmacológico , Adulto , Anciano , Ciclofosfamida/farmacología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Vidarabina/farmacologíaRESUMEN
We reviewed eight cases that were diagnosed before 1995 with B-prolymphocytic leukaemia (B-PLL) harbouring t(11;14)(q13;q32) and/or cyclin D1 staining. Thirteen B-PLL patients without t(11;14) were selected as controls. Peripheral blood, bone marrow and histological sections were re-examined without cytogenetic information. Final diagnosis was made using morphology, cytogenetics, immunophenotype and immunohistochemistry. Clinical characteristics were similar for both groups except for younger age, male predominance and extranodal involvement in cases with t(11;14). CD5 was more frequently positive in the t(11;14)+ group (80%) than in the t(11;14)- group (31%). Surface membrane immunoglobulin was strongly expressed by all t(11;14)+ cases, but only 45% of t(11;14)- cases. Histopathological and cytological review of cases with t(11;14) showed an infiltrate with a mixture of cells, some resembling prolymphocytes and others with mantle cell lymphoma (MCL) morphology. Blood films of cases with t(11;14) showed features suggestive of B-PLL in three, and in others, a mixture of cells resembling MCL and nucleolated ones; none corresponded to the blastoid form of MCL. We suggest that 'B-PLL' with t(11;14) may represent a splenomegalic form of MCL evolving with leukaemia. These cases illustrate the importance of tissue diagnosis with cyclin D1 staining and fluorescence in situ hybridization analysis in B-cell leukaemia with prolymphocytic features.
Asunto(s)
Cromosomas Humanos Par 11/genética , Cromosomas Humanos Par 14/genética , Leucemia de Células B/genética , Leucemia Prolinfocítica/genética , Translocación Genética , Anciano , Biomarcadores de Tumor/metabolismo , Ciclina D1/metabolismo , Diagnóstico Diferencial , Femenino , Humanos , Hibridación Fluorescente in Situ , Leucemia de Células B/diagnóstico , Leucemia de Células B/patología , Leucemia Prolinfocítica/diagnóstico , Leucemia Prolinfocítica/patología , Linfoma de Células del Manto/diagnóstico , Linfoma de Células del Manto/patología , Masculino , Persona de Mediana Edad , Proteínas de Neoplasias/metabolismo , Bazo/patología , Análisis de SupervivenciaRESUMEN
Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade B-cell lymphoma defined in the World Health Organization classification as the leukaemic form of splenic marginal zone lymphoma. Presenting features and response to therapy have been described, but information on prognostic factors is scanty. Clinical, laboratory and follow-up data were collected on 129 patients with SLVL to determine features predicting disease behaviour and survival. Diagnosis was made on clinical, morphological and immunophenotypic features and, where available, bone marrow and spleen histology. Median age was 69 years (range 39-90 years) and male:female ratio, 0.9. The majority had splenomegaly, but lymphadenopathy and hepatomegaly were rare. Median Hb was 11.8 g/dl, white blood cell count was 16 x 10(9)/l and platelet count was 145 x 10(9)/l; 27% of patients had monoclonal protein in serum and/or urine. While 27% of patients remained untreated, 10% transformed to high-grade lymphoma. Median follow-up was 61 months and median survival was 13 years, with 72% of patients alive at 5 years. Cox regression analysis showed that increasing age, anaemia, thrombocytopenia and lymphocytosis > 16 x 10(9)/l were independent adverse predictors of overall survival. However, only anaemia and lymphocytosis > 16 x 10(9)/l remained highly significant independent prognostic factors when only deaths due to lymphoma were analysed. Splenectomized patients fared better than those receiving chemotherapy only (P = 0.001 for SLVL deaths). We conclude that SLVL is mainly a disease of the elderly with a relatively benign course but, when treatment is required, splenectomy is beneficial.