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8.
Dermatologica ; 158(6): 397-403, 1979.
Artigo em Inglês | MEDLINE | ID: mdl-467758

RESUMO

Symptoms of the Sézary syndrome are described in a case history. Sézary's syndrome is a lymphoproliferative process of the skin, which eventually develops into an erythrodermia. Strange lymphocytes with a cerebriform chromatin structure, often with T-cell characteristics, circulate in the blood stream. In few instances they are also found in lymphnodes and bone marrow. This primarily benign disease can become malignant, possibly due to an overaggressive cytostatic therapy. Besides the hitherto unknown endogenous causes, exogenous factors cannot be excluded.


Assuntos
Síndrome de Sézary/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Sézary/sangue , Pele/patologia , Pele/ultraestrutura
9.
Schweiz Med Wochenschr ; 106(46): 1593-7, 1976 Nov 13.
Artigo em Alemão | MEDLINE | ID: mdl-138186

RESUMO

A case of the rare Sézary syndrome is described. The Sézary syndrome is a lymphoproliferative disorder of the skin, the lymph nodes, and to a smaller extent the bone marrow, which begins with erythrodermia. In the blood, strange circulating lymphocytes with a "cerebriform" chromatin structure and T-cell properties are found. The disease is primarly benign but may become malignant. The origin is unknown. Endogenic and exogenic factors are considered and relationships to other cutaneous lympho-proliferative diseases and treatment are discussed.


Assuntos
Doenças Linfáticas/tratamento farmacológico , Clorambucila/uso terapêutico , Dermatite Esfoliativa/tratamento farmacológico , Histiócitos , Humanos , Linfonodos/patologia , Linfócitos/patologia , Masculino , Pessoa de Meia-Idade , Plasmaferese , Prednisona/uso terapêutico , Síndrome
20.
J Clin Pathol ; 24(4): 354-9, 1971 May.
Artigo em Inglês | MEDLINE | ID: mdl-5556122

RESUMO

A family is described many of whose members suffered from renal insufficiency, hypertension, gout, and hyperuricaemia in conjunction. Adequate information was obtained on 72 subjects from five generations. In 17, one or more of the above mentioned abnormalities was or had been present. The hereditary distribution suggested an autosomal dominant disease entity. The renal disease was characterized by an early loss of urinary concentrating power, minimal proteinuria, and death at a relatively early age dominating the clinical picture. The histological picture in three biopsies and one necropsy showed predominant tubular atrophy and interstitial fibrosis, with striking tubular basement membrane thickening. It is suggested that these patients suffered from a hereditary degenerative renal disease. The question whether hyperuricaemia was primary or secondary in these cases is discussed.


Assuntos
Gota/genética , Hipertensão Renal/genética , Nefropatias/genética , Ácido Úrico/sangue , Adolescente , Adulto , Idoso , Biópsia , Transtornos Cerebrovasculares/complicações , Aberrações Cromossômicas , Transtornos Cromossômicos , Doença das Coronárias/complicações , Feminino , Gota/complicações , Humanos , Hipertensão Renal/complicações , Nefropatias/complicações , Testes de Função Renal , Masculino , Doenças Metabólicas/complicações , Doenças Metabólicas/genética , Pessoa de Meia-Idade , Infarto do Miocárdio/complicações , Uremia/complicações , Uremia/mortalidade , Uremia/patologia
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