RESUMO
So far the authors have observed 27 cases of accumulation of malignant haematologic diseases within a family in their county. They published the first ten cases in Orvosi Hetilap in 1992. Most often the co-existence of the diseases with the highest incidence (non-Hodgkin's lymphoma, chronic lymphocytic leukaemia, acute leukaemia) was observed, in terms of relationship mostly the parent--child combination was observed. Analyzing the data of parent--child combinations (17 cases) they can notice two remarkable trends: 1. The "malignity degree" of the disease appearing in the second generation is either the same or greater than that of the disease of the first generation (the opposite of this was not experienced in any of the cases!). 2. The disease of the second generation appears mostly at a much younger age than that of the parent. Consequently, they can observe a "double acceleration": the disease tends to be more malignant nearly in the half of the second generation patients, and the disease appears in the children at a much younger age.
Assuntos
Neoplasias Hematológicas/genética , Leucemia/genética , Linfoma/genética , Adolescente , Adulto , Criança , Feminino , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/epidemiologia , Humanos , Hungria/epidemiologia , Transmissão Vertical de Doenças Infecciosas , Leucemia/epidemiologia , Linfoma/epidemiologia , Masculino , Pessoa de Meia-IdadeRESUMO
The development of primary cutaneous immunoblastic lymphoma, then its leukaemic phase was observed by authors in the course of disease of the young man with primary cutaneous centrocytic lymphoma. Authors think this case counts on interest because of the unusual appearance and course of centrocytic lymphoma.
Assuntos
Linfoma não Hodgkin/patologia , Neoplasias Cutâneas/diagnóstico , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transformação Celular Neoplásica , Terapia Combinada , Humanos , Leucemia de Células B/patologia , Leucemia de Células B/fisiopatologia , Leucemia de Células B/terapia , Linfoma não Hodgkin/fisiopatologia , Linfoma não Hodgkin/terapia , Masculino , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapiaRESUMO
A case of a 40-year-old female is reported. Her symptoms started with abdominal complaints, loss of weight, general weakness, then gradually polyneuropathy, worsening hypotonia and systolic heart murmur developed. The bone marrow showed 20-25% infiltration by myeloma. An IgG lambda type paraproteinaemia was diagnosed using immunelectrophoresis. The ejection systolic murmur was caused by restrictive cardiomyopathy--characteristic of primary amyloidosis--with obstruction of the outflow tract of the left ventricle. Echocardiographic changes took 11 months to develop. The patient's status became dominated by severe hypotonia and she died as a result of heart failure. The post mortem examination confirmed amyloidosis with myeloma. Histopathological examination of the myocardium, coronary and other arteries revealed severe changes with AL-type amyloid deposits. The case demonstrates that amyloidosis can lead not only to restrictive cardiomyopathy but obstruction of the outflow tract of the left ventricle.
Assuntos
Amiloidose/complicações , Cardiomiopatia Dilatada/complicações , Mieloma Múltiplo/complicações , Adulto , Amiloidose/patologia , Medula Óssea/patologia , Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/patologia , Eletrocardiografia , Feminino , Humanos , Imunoglobulina G/imunologia , Mieloma Múltiplo/imunologia , Mieloma Múltiplo/patologia , Paraproteinemias/imunologiaRESUMO
Nine cases of familial malignant haematologic diseases were found by authors. Demonstrating the clinical pictures and the developments of nine pairs of cases (Hodgkin's disease--non-Hodgkin's lymphoma, Hodgkin's disease--chronic lymphocytic leukaemia, non-Hodgkin's lymphoma--acute lymphoblastic leukaemia, hairy cell leukaemia--acute lymphoblastic leukaemia, chronic lymphocytic leukaemia--acute myelogenous leukaemia, non-Hodgkin's lymphoma--chronic lymphocytic leukaemia, and three times chronic lymphocytic leukaemia--chronic lymphocytic leukaemia) authors want to give data about occurrences of familial leukaemia/lymphoma in their county.