| Objective To investigate etiological factors, pathogenesis, clinical manifestation, laboratory examination, histopathological features and diagnostic evidence of primary effusion lymphoma (PEL). Methods The clinical manifestation and laboratory data of a patient with AIDS associated PEL and nervous system lesion were reviewed. The biopsy and autopsy specimens were studied by hematoxylin-eosin staining, immunohistochemical staining and polymerase chain reaction(PCR). Results The patient was a 23-year-old female. The clinical manifestation were characterized by 13 months progressive course, serous liquid growth in multiple serous membrane cavities(~pleura, pericardium, peritoneum), countinuous superficial lymph node tumefaction of total body, intermission low fever, loss of weight and anemia. 14 days after admission , she died of cardiac taniponade. Histopathological study showed neoplastic lymphocytes infiltration of multiple organs, particular of lung membrane, epicardium, part of peritoneum and bone marrow. Lymphatic follicles in whole body lymph nodes and spleen were nearly extincted. In remaining T field ,the number of lymphocyte apparently decreased, and small vessels proliferated actively. Many neoplastic lymphocytes,especially those large, round cells showed immunoblastic features ,which contained abundant basophilic cytoplasm and large, round nuclei with prominent nucleoli. Immunohistochemical staining revealed that the homogeneous 2population of large cells were CD43 and CD3 positive and CD2O negative. PCR demonstrated I-IIV DNA in the retroperitoneal lymph node. The final diagnoses were AIDS associated PEL, cerebral vascular disease and myopathy. Conclusion PEL represent a rare subset of lymphomas ,which was closely related to human herpesvirus 8(HHV-8) infection and mostly occurred in patients with AIDS. The immunophenotypic markers of malignant monoclone were extremly rare T cell phenotype . Only 2 cases has been reported in the world.
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