Adult ; Female ; Humans ; Lymphoma, T-Cell ; pathology ; physiopathology ; Lymphoma, T-Cell, Cutaneous ; pathology ; physiopathology ; Myositis ; physiopathology

Adult ; Female ; Humans ; Lymphoma, T-Cell ; pathology ; physiopathology ; Panniculitis ; physiopathology ; Subcutaneous Tissue ; pathology ; physiopathology

Intravascular lymphomatosis (IL) is a rare and generally fatal disease characterized by proliferation of large lymphoma cells almost exclusively within the lumen of small blood vessels. The skin and central nervous systems are typically affected, but involvement of other organs, such as lung, has been described. Predominant lung involvement without cutaneous and neurologic manifestation is very rare and difficult to diagnose. Originally considered as an endothelial disorder, IL has recently been reclassified as lymphoma. Most of the cases reported are of B cell lineage with a few cases of T cell type. We describe a case of the T-cell type IL manifested clinically as an interstitial lung disease without involvement of skin and central nervous systems. Immunohistochemical studies showed the T-cell nature of the neoplastic cells in open lung biopsy sample.
Blood Vessels/pathology ; Case Report ; Diagnosis, Differential ; Fatal Outcome ; Human ; Lung Diseases, Interstitial/pathology* ; Lymphoma, T-Cell/physiopathology ; Lymphoma, T-Cell/pathology* ; Lymphoma, T-Cell/immunology ; Male ; Middle Age ; Tomography Scanners, X-Ray Computed

As the chemotherapeutic resistance of extranodal NK/T-cell lymphoma (ENKTL) rises year by year, searching for novel chemoprevention compounds has become imminent. Gambogic acid (GA) has recently been shown to have anti-tumor effects, but its role and underling mechanism in ENKTL are rather elusive. In the present study, we showed that GA inhibited the cell growth and potently induced the apoptosis of ENKTL cells in vitro in a time- and concentration-dependent manner. Furthermore, GA induced cell death through endoplasmic reticulum stress (ERS) mediated suppression of Akt signaling pathways and finally the release of the caspase-3 proteases. Overall, our data provided evidences supporting GA as a potential therapeutic agent for ENKTL, which may facilitate further preclinical development of anti-tumor drugs.
Apoptosis ; drug effects ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Endoplasmic Reticulum Stress ; drug effects ; Humans ; Lymphoma, Extranodal NK-T-Cell ; drug therapy ; genetics ; metabolism ; physiopathology ; Proto-Oncogene Proteins c-akt ; genetics ; metabolism ; Signal Transduction ; drug effects ; Xanthones ; pharmacology