抄録
A case of lymphoblastic lymphoma with high terminal transferase (TdT) activity in the tumor cells was reported, which showed frequent infiltrations in various organs and sites.
The patient, a 41-year-old man was admitted because of cervical and axillary lymph node swelling, and showed a large mediastinal mass and a massive pleural effusion in the X-ray films of the chest. Microscopical examination of the lymph node revealed a diffuse infiltration of uniform blastic cells with scanty cytoplasm and round or indented nuclei. Frequent mitotic figures and scattered starry-sky patterns were present. Localized positive granule for acid phosphatase stain was detected in the cytoplasm in the touch imprint of the specimen.
Histological diagnosis was malignant lymphoma, poorly differentiated lymphocytic, diffuse (PDL-D), and VEPA therapy was started. After 2 weeks complete remission was achieved, however CNS involvement developed immediately, and then frequent infiltrations appeared in CNS, neck muscle, CNS, bone marrow, skin and ascites. Eleven months after admission he died of leukoencephalopathy which seemed to be induced by cranial radiation and intrathecal chemotherapy with MTX and Ara-c etc.
Clinical pictures, cytological findings and high levels of TdT, which were detected in the tumor cells of lymph node, pleural effusion, bone marrow and ascites, indicated that the case was lymphoblastic lymphoma (convoluted cell type) that has been included in PDL-D. Although no surface markers were demostrated in the lypmh node cells, E-rosette formation was detected in the tumor cells of ascites.
The relationship between tumor cell origin and clinical pictures and therapy was discussed.