title: Peripheral T-cell lymphoma presenting with rapidly progressing myelofibrosis.
abstract: 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.
authors.affiliation: First Department of Internal Medicine, Kagawa Medical University, 1750-1, Ikenobe, Miki-cho, Kagawa, 761-0793 Japan.
authors.forename: Eisuke
authors.initials: E
authors.lastname: Uehara
authors.forename: Taizo
authors.initials: T
authors.lastname: Tasaka
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Matsuhashi
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Fujita
authors.forename: Takahiro
authors.initials: T
authors.lastname: Tamura
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Shimoura
authors.forename: Shohei
authors.initials: S
authors.lastname: Mano
authors.forename: Minoru
authors.initials: M
authors.lastname: Kuwajima
authors.forename: Masami
authors.initials: M
authors.lastname: Nagai
mesh_terms: D000368:Aged; D000740:Anemia / Q000209:etiology; D017809:Fatal Outcome; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D016411:Lymphoma, T-Cell, Peripheral / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D055728:Primary Myelofibrosis / Q000209:etiology*; D013921:Thrombocytopenia / Q000209:etiology; D016212:Transforming Growth Factor beta / Q000097:blood
chemical_list: D016212:Transforming Growth Factor beta