Peripheral T-cell lymphoma presenting with rapidly progressing myelofibrosis.
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.
Uehara
Eisuke
E
First Department of Internal Medicine, Kagawa Medical University, 1750-1, Ikenobe, Miki-cho, Kagawa, 761-0793 Japan.
Tasaka
Taizo
T
Matsuhashi
Yoshiko
Y
Fujita
Mitsuhiro
M
Tamura
Takahiro
T
Shimoura
Yasumasa
Y
Mano
Shohei
S
Kuwajima
Minoru
M
Nagai
Masami
M
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
D016212:Transforming Growth Factor beta