ABSTRACT
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.
Subject(s)
Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/drug therapy , Adult , Algorithms , Ascorbic Acid/administration & dosage , Bone Marrow/pathology , Child , Combined Modality Therapy , DNA Mutational Analysis , Diagnosis, Differential , Early Diagnosis , Female , Histamine H1 Antagonists/therapeutic use , Humans , Infant, Newborn , Ketotifen/administration & dosage , Mast Cells/pathology , Mastocytosis, Systemic/classification , Mastocytosis, Systemic/genetics , Pregnancy , Proto-Oncogene Proteins c-kit/genetics , RNA, Messenger/genetics , Ranitidine/administration & dosageABSTRACT
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.
Subject(s)
Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Lung Neoplasms/secondary , Neoplasms, Second Primary/immunology , Pleural Effusion/etiology , Antigens, CD , Humans , Lung Neoplasms/immunology , Lung Neoplasms/pathology , Lymph Nodes/immunology , Lymph Nodes/pathology , Lymphocytes/pathology , Male , Middle Aged , Neoplasms, Second Primary/pathologyABSTRACT
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.
