RESUMO
In the present paper clinical phenotypes, pathogenetic relationships, and diagnostic algorithms as well as therapeutic concepts of/for systemic mast cell activation disease are reviewed. The reader should be able to recognize and diagnose a systemic mast cell activation disease, as well as to counsel a personalized drug therapy. In the case of chronic multisystem polymorbidity systemic mast cell activation disease should be considered as a differential diagnosis at an early stage. In most cases, specific, little invasive investigations allow diagnosing the disease and, hence, an appropriate therapy can be initiated.
Assuntos
Mastocitose Sistêmica/diagnóstico , Mastocitose Sistêmica/tratamento farmacológico , Adulto , Algoritmos , Ácido Ascórbico/administração & dosagem , Medula Óssea/patologia , Criança , Terapia Combinada , Análise Mutacional de DNA , Diagnóstico Diferencial , Diagnóstico Precoce , Feminino , Antagonistas dos Receptores Histamínicos H1/uso terapêutico , Humanos , Recém-Nascido , Cetotifeno/administração & dosagem , Mastócitos/patologia , Mastocitose Sistêmica/classificação , Mastocitose Sistêmica/genética , Gravidez , Proteínas Proto-Oncogênicas c-kit/genética , RNA Mensageiro/genética , Ranitidina/administração & dosagemRESUMO
We report the case of a patient with a chronic lymphocytic leukemia (CLL) who later developed a metastasized large-cell neuroendocrine carcinoma of the lung that was complicated by a malignant pleural effusion. In contrast to the peripheral blood where the malignant B-CLL cells represented >99% of all lymphocytes, lymphocytes infiltrating the malignant effusion were mainly T cells. Nearly all of these T cells were CD4(+). This stood in sharp contrast to the peripheral blood where the CD4(+)/CD8(+) ratio remained balanced. A detailed analysis of the CD4(+) T cells within the malignant effusion revealed that these cells uniformly expressed a CCR7(+) CD62L(+) "non-effector" phenotype. When the monoclonal B cells within the malignant effusion were analyzed, we found that these cells, in contrast to the B-CLL cells in the peripheral blood, were negative for CD23 and expressed much higher levels of the adhesion molecules L-selectin (CD62L) and CD11a. A deficient expression of these adhesion molecules might have led to a "trapping" of the majority of B-CLL cells in the peripheral blood. This phenomenon might have contributed to the development of two highly different immunological compartments in this patient with CLL and pleural effusion of a solid tumor.
Assuntos
Leucemia Linfocítica Crônica de Células B/patologia , Neoplasias Pulmonares/secundário , Segunda Neoplasia Primária/imunologia , Derrame Pleural/etiologia , Antígenos CD , Humanos , Neoplasias Pulmonares/imunologia , Neoplasias Pulmonares/patologia , Linfonodos/imunologia , Linfonodos/patologia , Linfócitos/patologia , Masculino , Pessoa de Meia-Idade , Segunda Neoplasia Primária/patologiaRESUMO
The effects of ultraviolet B (UV-B) irradiation on transmission of the rat cornea were studied in combination with or without naphthalene feeding. UV irradiation as well as naphthalene feeding caused a decrease in transmission in the wavelength region from 315 to 450 nm, UV irradiation being thereby the more hazardous influence. The combination of both noxae did not lead to a pronounced decrease as compared with UV irradiation alone. Although the histological findings of the eye lens suggest a syncataractogenic action of UV-B and naphthalene feeding, a corresponding effect could not be found by means of Scheimpflug photography. This could be possibly due to the alteration in corneal transmission properties following UV-B irradiation.
