transforming-growth-factor-beta and Lymphoma--T-Cell--Peripheral

Myelofibrosis is usually observed in myeloproliferative disorders, such as chronic myeloid leukemia. However, there are only a few reports showing an association between T-cell lymphoma and myelofibrosis. We report a case of peripheral T-cell lymphoma, unspecified (diffuse large cell) type, involving the bone marrow that was associated with severe myelofibrosis. In the present case, the plasma concentration of transforming growth factor-beta1 (TGF-beta1) was increased to 8.95 ng/ml (normal range: 1.56-3.24 ng/ml). No lymphadenopathy or skin lesions were observed during the entire clinical course. Although the mechanism of secondary myelofibrosis is still unclear, elevated plasma TGF-beta1 might be involved in the pathogenesis of bone marrow fibrosis in the present case.

Topics: Aged; Bone Marrow; Fatal Outcome; Humans; Lymphoma, T-Cell, Peripheral; Male; Primary Myelofibrosis; Transforming Growth Factor beta; Transforming Growth Factor beta1

Myelofibrosis following peripheral T-cell lymphoma has rarely been reported. Described here is a case of peripheral T-cell lymphoma with myelofibrosis and elevated transforming growth factor beta (TGF-beta). A 69 years old male was admitted due to anemia and thrombocytopenia. His bone marrow showed fibrosis and was infiltrated with small lymphoid cells and a few residual normal hematopoietic cells. He had presented with hepatosplenomegaly and left inguinal lymph node swelling. Biopsy of the left inguinal lymph node revealed diffuse mature small lymphoid cells with atypical nuclei. Immunophenotyping of the small lymphoid cells were positive for CD3, CD8, TCR alphabeta and HLA-DR and were negative for CD4, CD19, CD20 and CD56. T-cell receptor beta-chain gene was rearranged in bone marrow cells. He was diagnosed as having peripheral T-cell lymphoma complicated with myelofibrosis. Chemotherapy was administrated which improved his pancytopenia and symptoms. Two years later, anemia and thrombocytopenia developed rather quickly, he died because of progression of myelofibrosis with severe pancytopenia.

Topics: Aged; Anemia; Fatal Outcome; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; Humans; Immunophenotyping; Lymphoma, T-Cell, Peripheral; Male; Primary Myelofibrosis; Thrombocytopenia; Transforming Growth Factor beta

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Cyclophosphamide; Doxorubicin; Female; Hematopoietic Stem Cell Transplantation; Humans; Immunophenotyping; Lymphoma, T-Cell, Peripheral; Neoplasm Proteins; Pancytopenia; Platelet-Derived Growth Factor; Prednisolone; Primary Myelofibrosis; Remission Induction; T-Lymphocytes, Cytotoxic; Transforming Growth Factor beta; Transplantation, Homologous; Vincristine