RESUMO
We report three family members, including a set of identical twins, who developed CD5 positive B-CLL. The patients are female Ashkenazi Jews sharing an identical HLA phenotype. Two of the HLA loci (B35 and Cw4) were common with those already described as being shared by Ashkenazi Jews with an increased incidence of CLL. The rearranged immunoglobulin genes of the malignant cells of all three patients were found to express genetically related VH regions belonging to the VH3 subgroup, and chromosomal studies suggest a process of clonal evolution in one of the twins.
Assuntos
Antígenos CD5 , Rearranjo Gênico de Cadeia Pesada de Linfócito B , Genes de Imunoglobulinas , Leucemia Linfocítica Crônica de Células B/genética , Idoso , Sequência de Bases , Primers do DNA/genética , Feminino , Antígenos HLA , Teste de Histocompatibilidade , Humanos , Israel , Judeus , Leucemia Linfocítica Crônica de Células B/etnologia , Leucemia Linfocítica Crônica de Células B/imunologia , Pessoa de Meia-Idade , Dados de Sequência Molecular , Fenótipo , Reação em Cadeia da Polimerase , Risco , Gêmeos MonozigóticosRESUMO
Sixty-three patients with Hodgkin's disease, in stages I or II, asymptomatic (A) or symptomatic (B), were diagnosed and followed at the Chaim Sheba Medical Center from 1969 to 1976. Only 14 were staged pathologically. Until 1971, the patients received mantle or "inverted Y" therapy only; thereafter, an extended field that included mantle, upper abdomen and spleen irradiation was given. Symptomatic patients, as well as patients with extranodal involvement, received MOPP chemotherapy (nitrogen mustard, vincristine, procarbazine and prednisone) after termination of radiotherapy. Of 51 patients who were in stage IA or IIA, six relapsed 20 to 43 months after irradiation. Three had a pelvic recurrence; two of them were surgically staged. Thus, in only 1 of 51 patients could staging laparotomy possibly have detected pelvic disease and resulted in different therapy. Our results suggest that total nodal irradiation and staging laparotomy are not mandatory in stages IA and IIA of Hodgkin's disease. The group of 12 symptomatic patients is too small to allow us to draw definite conclusions as to the role of staging laparotomy and adjuvant chemotherapy. However, in view of the high relapse rate in the upstaged symptomatic patients, it seems that chemotherapy should be given to these patients.
Assuntos
Doença de Hodgkin/terapia , Esplenectomia , Adolescente , Adulto , Criança , Feminino , Doença de Hodgkin/patologia , Doença de Hodgkin/radioterapia , Humanos , Masculino , Mecloretamina/uso terapêutico , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prednisona/uso terapêutico , Procarbazina/uso terapêutico , Vincristina/uso terapêuticoRESUMO
Primary intestinal lymphoma in young adults is a disease that occurs mainly in underprivileged populations. There is evidence that in some cases this disease evolves from a benign lymphoplasmocytic infiltration of the gut with alpha heavy chain. More studies are needed on the effect of environmental and genetic factors on the evolution of this disease. The role of oncogenic viruses in the development of intestinal lymphoma with malabsorption is an open question. Regional studies on the entity of intestinal lymphoma with malabsorption and its relationship to childhood lymphoma in the same populations are warranted.
