title: Angioimmunoblastic T-cell lymphoma with intramedullary production of platelet-derived growth factor and possibly complicating myelofibrosis: report of a case with review of the literature.
abstract: A 65-year-old man was diagnosed with angioimmunoblastic T-cell lymphoma (AITL) with bone marrow (BM) infiltration and myelofibrosis (MF). The BM infiltration and the condition of the MF improved following CHOP therapy (cyclophosphamide hydrate, doxorubicin hydrochloride, vincristine sulfate, and prednisolone). After complete remission was achieved, early central nervous system recurrence was noted, with no evidence of BM infiltration or MF. The lymph nodes and BM were examined for cytokines by immunohistochemical staining with monoclonal murine antibodies. The lymphoma cells were positive only for platelet-derived growth factor (PDGF) and negative for basic fibroblast growth factor, fibronectin, vascular endothelial growth factor, transforming growth factor-β (TGF-β), tumor necrosis factor α, interleukin-1β, and interleukin-6. It was thus inferred that the lymphoma cells producing PDGF caused the MF, and that the absence of MF at relapse may have been attributable to the absence of BM infiltration. There have been seven reported cases of AITL with intercurrent MF, although cytokine data (elevations of blood PDGF and TGFβ levels) are available for only one case. The present report is to our knowledge the only report of a case of AITL complicated by MF for which the results of immunohistostaining with anticytokine antibodies are available.
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba Prefecture, Japan. yasu_sek@juntendo-urayasu.jp
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Sekiguchi
authors.forename: Nana
authors.initials: N
authors.lastname: Matsuzawa
authors.forename: Asami
authors.initials: A
authors.lastname: Shimada
authors.forename: Hidenori
authors.initials: H
authors.lastname: Imai
authors.forename: Mutsumi
authors.initials: M
authors.lastname: Wakabayashi
authors.forename: Keiji
authors.initials: K
authors.lastname: Sugimoto
authors.forename: Noriko
authors.initials: N
authors.lastname: Nakamura
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Sawada
authors.forename: Kouji
authors.initials: K
authors.lastname: Izutsu
authors.forename: Kengo
authors.initials: K
authors.lastname: Takeuchi
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ohta
authors.forename: Norio
authors.initials: N
authors.lastname: Komatsu
authors.forename: Masaaki
authors.initials: M
authors.lastname: Noguchi
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage; D016207:Cytokines / Q000378:metabolism; D004317:Doxorubicin / Q000008:administration & dosage; D006801:Humans; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D008297:Male; D009363:Neoplasm Proteins / Q000096:biosynthesis*; D010982:Platelet-Derived Growth Factor / Q000096:biosynthesis*; D011241:Prednisone / Q000008:administration & dosage; D055728:Primary Myelofibrosis / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D016207:Cytokines; D009363:Neoplasm Proteins; D010982:Platelet-Derived Growth Factor; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone