Assuntos
Linfócitos B/patologia , Linfocitose/epidemiologia , Adolescente , Adulto , Ásia/epidemiologia , Criança , Pré-Escolar , Células Clonais/patologia , Comorbidade , Europa (Continente)/epidemiologia , Rearranjo Gênico do Linfócito B , Predisposição Genética para Doença , Antígeno HLA-DR7/genética , Humanos , Imunoglobulinas/biossíntese , Imunoglobulinas/genética , Japão/epidemiologia , Leucemia de Células Pilosas/classificação , Leucemia de Células Pilosas/epidemiologia , Leucemia de Células Pilosas/patologia , Linfocitose/classificação , Linfocitose/patologia , Transtornos Linfoproliferativos/classificação , Transtornos Linfoproliferativos/epidemiologia , Transtornos Linfoproliferativos/patologia , Fumar/efeitos adversos , Fumar/epidemiologia , Estados Unidos/epidemiologiaAssuntos
Leucemia Mielogênica Crônica BCR-ABL Positiva/classificação , Medula Óssea/patologia , Doenças da Medula Óssea/classificação , Diferenciação Celular , Transformação Celular Neoplásica/genética , Progressão da Doença , Proteínas de Fusão bcr-abl/análise , Proteínas de Fusão bcr-abl/genética , Células-Tronco Hematopoéticas/patologia , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologia , Transtornos Mieloproliferativos/classificação , Células-Tronco Neoplásicas/patologia , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/classificação , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologiaAssuntos
Policitemia Vera/terapia , Doença Aguda , Alquilantes/uso terapêutico , Bussulfano/uso terapêutico , Clorambucila/efeitos adversos , Clorambucila/uso terapêutico , Humanos , Hidroxiureia/uso terapêutico , Leucemia Mieloide/induzido quimicamente , Leucemia Mieloide/epidemiologia , Leucemia Induzida por Radiação/induzido quimicamente , Leucemia Induzida por Radiação/epidemiologia , Flebotomia , Radioisótopos de Fósforo/efeitos adversos , Radioisótopos de Fósforo/uso terapêutico , Pipobromano/uso terapêutico , Inibidores da Agregação Plaquetária/uso terapêutico , Policitemia Vera/tratamento farmacológico , Policitemia Vera/mortalidade , Policitemia Vera/radioterapia , Ensaios Clínicos Controlados Aleatórios como Assunto , Taxa de SobrevidaRESUMO
PURPOSE: 1. To recognise the clinico-biological profile of a group of patients diagnosed of polycythaemia vera (PV) in our centre in the last 30 years. 2. To identify the evolutive patterns of haematological transformation. 3. To evaluate the effect of therapy on the survival. PATIENTS AND METHODS: The clinical records of 74 patients (median age 62 years, male/female = 0.94, followed-up for 6-357 months, median 64 months) were reviewed. Clinico-biological data at diagnosis, therapy, complications and evolution of the haematological picture were evaluated in each case. The actuarial survival in the series was compared to that of the normal population. RESULTS: The clinico-analytical data and diagnostic features were identical to other series reported. Mild increases of bone marrow reticulin was present in two thirds of the cases, overt myelofibrosis being found in only 10% of the patients. Abnormal karyotype was seen in 9% of the patients (11q-, -Y). Phlebotomy was the only treatment in eight cases, without increased incidence of thrombotic phenomena. The remainders received myelosuppressive therapy (32P, busulphan, pipobroman, hydroxyurea, etc.), thrombotic complications appearing in 8 cases and haemorrhagic complications in 4 others. One of these latter patients developed oesophageal carcinoma. The haematological picture evolved into toxic aplastic anaemia in 2 cases; myelofibrosis with myeloid metaplasia (MF/MM) in 8; myelodysplastic sindromes (MDS) in 5, three of them RAEB; and acute myelogenous leukaemia in 3 cases, two of them as the final stage of previous MF/MM and MDS/ RAEB. The actuarial survival was 71% at ten years and 46% at fifteen years, and the median survival as a whole was 13.5 years. CONCLUSIONS: 1: The treatment, mostly myelosuppressive, given to these patients attained a survival similar to that of the general population. 2: Of the cases with known evolution, 15.6% developed MF/MM, its incidence being higher in patients treated only with phlebotomy (37%). 3: The incidence of malignant evolution, i.e., to RAEB/AML, amongst those patients followed-up was 10.6%.
Assuntos
Medula Óssea/patologia , Células Clonais/patologia , Policitemia Vera/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Policitemia Vera/diagnóstico , Policitemia Vera/tratamento farmacológico , Policitemia Vera/mortalidade , Policitemia Vera/terapia , Estudos Retrospectivos , Análise de SobrevidaAssuntos
Linfoma de Células B , Linfoma não Hodgkin , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Medula Óssea , Terapia Combinada , História do Século XX , Humanos , Fatores Imunológicos/uso terapêutico , Linfoma de Células B/classificação , Linfoma de Células B/história , Linfoma de Células B/patologia , Linfoma de Células B/terapia , Linfoma não Hodgkin/classificação , Linfoma não Hodgkin/história , Linfoma não Hodgkin/patologia , Linfoma não Hodgkin/terapia , Estadiamento de Neoplasias , Teleterapia por Radioisótopo , Indução de RemissãoAssuntos
Linfoma não Hodgkin/patologia , Neoplasias do Mediastino/patologia , Adolescente , Adulto , Criança , Terapia Combinada , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Recém-Nascido , Linfoma de Células B/diagnóstico , Linfoma de Células B/patologia , Linfoma de Células B/terapia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/terapia , Masculino , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/terapia , Mediastino/patologia , Pessoa de Meia-Idade , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologiaAssuntos
Compartimento Celular/fisiologia , Tecido Linfoide/fisiopatologia , Linfoma não Hodgkin/fisiopatologia , Linfócitos B/patologia , Linfócitos B/fisiologia , Humanos , Tecido Linfoide/imunologia , Tecido Linfoide/patologia , Linfoma não Hodgkin/imunologia , Linfoma não Hodgkin/patologia , FenótipoRESUMO
BACKGROUND: Immunoproliferative small intestine disease (IPSID) encompasses a primary intestinal lymphoma of underpriviliged populations of North Africa, Middle East, etc. This epidemiological feature strongly implicates environmental and host (genetic) factors in its pathogenesis. IPSID can be distinguished on clinicopathological grounds from "Western-type" intestinal lymphomas. "IPSID-like" lymphomas had been sporadically identified, i.e., patients with original clinico-analytical data of IPSID (chronic diarrhoea, malabsortion, clubbing of fingers, diffuse intestinal involvement, etc.) without its conventional histopathologic (lymphoplasmocytic or plasmocytic infiltration) and immunological (alpha-heavy-chain paraprotein) background. PURPOSE: The aim of this study has been: 1) to identify, in a series of small intestine lymphomas, a group of patients with a long-lasting history of chronic diarrhoea and a clinico-biologic pattern of "IPSID-like" lymphoma; 2) to analyze its clinicopathological profile; 3) to search for differences with the pattern of the remaining cases ("Western-type" lymphomas) and 4) To suggest a possible epidemiological significance. PATIENTS AND METHODS: Patients considered were 12 Spanish caucasians with primary intestinal lymphoma and a long-lasting history of chronic diarrhoea vs 31 cases of "Western-type" intestinal lymphomas admitted in our Hospital over a 33-year period. Statistical significance of differences in clinico-biological features (symptoms/signs, analytical data, patterns of involvement, histopathology, immunophenotype and tumor staging) between these two groups was evaluated using X2 test. RESULTS: The results of this retrospective study allow us to delineate a relatively homogeneous "IPSID-like" group (12 cases) among 43 cases of primary small intestine lymphoma diagnosed between 1960 and 1993. The clinico-pathological behavior of these patients was significantly different from that exhibited by the 31 cases of so-called "Western-type" lymphomas. CONCLUSIONS: It is suggested that they may represent a group of patients suffering an evanescent "IPSID-equivalent" disorder (last case diagnosed in 1975), that probably has evolved in similar but not identical epidemiological circumstances to those present in the "Third World" countries of our Mediterranean area.
Assuntos
Doença Imunoproliferativa do Intestino Delgado/epidemiologia , Neoplasias Intestinais/epidemiologia , Linfoma não Hodgkin/epidemiologia , Adolescente , Adulto , Idoso , Pré-Escolar , Doença Crônica , Países em Desenvolvimento , Diagnóstico Diferencial , Diarreia/etiologia , Suscetibilidade a Doenças/etnologia , Meio Ambiente , Feminino , Antígenos HLA/análise , Humanos , Doença Imunoproliferativa do Intestino Delgado/complicações , Doença Imunoproliferativa do Intestino Delgado/diagnóstico , Neoplasias Intestinais/complicações , Neoplasias Intestinais/diagnóstico , Linfoma de Zona Marginal Tipo Células B/classificação , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Linfoma não Hodgkin/classificação , Linfoma não Hodgkin/diagnóstico , Masculino , Região do Mediterrâneo/epidemiologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores Socioeconômicos , Espanha/epidemiologia , População BrancaAssuntos
Gastroenteropatias/complicações , Doenças Hematológicas/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia/etiologia , Anemia Perniciosa/etiologia , Criança , Doença Crônica , Eritropoese , Feminino , Gastrite Atrófica/complicações , Gastroenteropatias/diagnóstico , Hemorragia Gastrointestinal/complicações , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiologia , Doenças Hematológicas/diagnóstico , Humanos , Ferro/metabolismo , Síndromes de Malabsorção/complicações , Síndromes de Malabsorção/etiologia , MasculinoRESUMO
PURPOSE: To compare the maximum epidemiologic data attained from myelodysplastic syndromes (MDS) with those of two main panmyelopathies, namely acute myeloblastic leukaemia (AML) and aplastic pancytopenia (AP). PATIENTS AND METHODS: A retrospective analysis was carried out on 21,135 patients included in the Bone Marrow Study Registry of the Jiménez Díaz Foundation along 35 years (1959-1993). The data were grouped into seven five-year periods. Of these, in the first three the study was performed on bone-marrow aspirates; after 1976 the histopathological study of bone-marrow biopsies was introduced, and since 1979 the karyotype has been regularly examined. The MDS were classified in accordance with the FAB system. With these premises borne in mind, the following aspects were considered: diagnostic interpretation of MDS along the years; diagnostic incidence of MDS, AML and AP in each of the five-year periods; relative frequency of those diagnosis with respect to the total number of cases; evolutive profile of sex and age at diagnosis; quantitative significance of secondary MDS-AL and toxic AP along the years; MDS subtypes and their epidemiologic characteristics. RESULTS: A total of 510 MDS, 610 AML and 223 AP cases were identified. With respect to the sex of the MDS patients, some changes have been seen along the years, from an M/F ratio of 1.9 to 1.0; and the mean age at diagnosis raised from 53.3 years (with only 1.7% of the cases over 65 years of age) to 71.4 years (with 76.9% of the cases over 65 years of age), all this within the 1959-1989 period. The incidence of AML and AP has remained stable for the last 20 years; on the contrary, MDS have been increasing continuously along the 35 years of the study, which poses for a higher number of new cases in every period (from 35 to 119) and also for a higher relative frequency in the registry (from 1.37% to 4.40%) within the period 1959-1989. Valuable toxic history was progressively increasing in secondary MDS-AML and progressively decreasing in AP. With respect to the FAB subtypes of MDS, and taking into account the last of the five-year periods, the most frequently diagnosed were RA and RSA followed by RAEB, CMML and RAEB-T. CONCLUSIONS: The increment of the incidence of MDS cases correlates significantly with the increment of the patients aged over 65 years. This incidence appears to be scarcely influenced by previous mutagenic agents (radiotherapy, chemotherapy) and might be due to a better understanding of MDS.
Assuntos
Leucemia Mieloide/epidemiologia , Síndromes Mielodisplásicas/epidemiologia , Doença Aguda , Distribuição por Idade , Idoso , Anemia Refratária/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Distribuição por Sexo , Espanha/epidemiologiaAssuntos
Linfoma de Zona Marginal Tipo Células B/classificação , Linfócitos B , Humanos , Mucosa Intestinal/citologia , Mucosa Intestinal/fisiologia , Tecido Linfoide/citologia , Tecido Linfoide/fisiologia , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/imunologia , Nódulos Linfáticos Agregados/citologia , Terminologia como AssuntoRESUMO
Five cases of idiopathic thrombocytopenic purpura (ITP) appearing on five members of two generations of a family, with autosomal dominant pattern, are presented. The clinico-biologic behaviour of 2 patients (studied and treated by the authors) plus the available data from the 3 others (diagnosed and treated at other hospitals) allowed us to discard any possibility of hereditary non-immunologic thrombocytopenia. The predisposition of ITP patients and their relatives to present clinico-biological features of autoimmune diseases is commented as a possible explanation for the rare forms of familial ITP.
Assuntos
Doenças Autoimunes/genética , Púrpura Trombocitopênica Idiopática/genética , Adolescente , Adulto , Pré-Escolar , Feminino , Genes Dominantes , Predisposição Genética para Doença , Humanos , Masculino , Pessoa de Meia-Idade , LinhagemAssuntos
Leucemia Mieloide Aguda/tratamento farmacológico , Síndromes Mielodisplásicas/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Humanos , Leucemia Mieloide Aguda/etiologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/complicações , Recidiva , Indução de Remissão , Fatores de TempoAssuntos
Genes ras , Leucemia Promielocítica Aguda/genética , Proteínas de Neoplasias , Proteínas Nucleares , Receptores do Ácido Retinoico/genética , Fatores de Transcrição/genética , DNA de Neoplasias/genética , Rearranjo Gênico , Humanos , Mutação Puntual , Proteína da Leucemia Promielocítica , Translocação Genética , Proteínas Supressoras de TumorRESUMO
PURPOSES: To analyze the reproducibility to detect the t (15; 17) with molecular and cytogenetic techniques. PATIENTS AND METHODS: Fifteen cases of acute promyelocytic leukaemia were studied by means of cytogenetic techniques (GTG banding) and molecular ones (K/S probe and HindIII, EcoRI and BamHI restriction enzymes). RESULTS: The t (15; 17) was detected with cytogenetic techniques in 60% cases and with molecular ones in 33% cases. Using both (cytogenetic and molecular) techniques the translocation was detected in 73% cases. CONCLUSIONS: The use of both techniques may be very useful in order to get a better diagnostic and a closer control of the patient's clinical evolution.
Assuntos
Cromossomos Humanos Par 15/ultraestrutura , Cromossomos Humanos Par 17/ultraestrutura , Leucemia Promielocítica Aguda/genética , Translocação Genética , Bandeamento Cromossômico , DNA de Neoplasias/genética , Humanos , Leucemia Promielocítica Aguda/patologia , Polimorfismo de Fragmento de Restrição , Reprodutibilidade dos TestesRESUMO
Myelodysplastic syndromes represent an entire group of clonal panmyelopathies with very distinct evolutionary pathways. Their common denominator, however, is a self-maintained functional failure of the myeloid hemopoiesis which tends to evolve into severe non-lymphoid leukemia (SNLL) in 20-30% of the cases. First, the prognostic value of each the following is reviewed: the morphological classification F.A.B., the stratification system for "Bournemouth group", the abnormal placement of immature myeloid precursors (ALIP) in bone marrow, and cytogenetic changes. Second, the therapeutic potential for each of the following is assessed: vitamin and support treatments; suprarenal steroids; conventional androgens and danazol; agents of cellular differentiation (cytosine arabinoside in low doses, retinoid acids, vitamin D3, etc.). Finally, the role of aggressive chemotherapies (in succession or unrelated to marrow transplant) in the eradication of myelodysplastic clone or post-myelodysplasia SNLL is examined.