title: Clinicopathologic and therapeutic aspects of angioimmunoblastic lymphadenopathy-related lesions.
abstract: The clinicopathologic features of 14 patients with angioimmunoblastic lymphadenopathy (AIL)-related lesions were analyzed. Lymph node biopsy specimens from all the patients showed a diffuse obliteration of lymph node architecture, prominent vascular proliferation, a polymorphous cellular infiltrate, including immunoblasts, and varying degrees of clear cell proliferation. The patients were eight males and six females, with a median age of 58.5 years. All but one were in an advanced stage at the time of diagnosis. Bone marrow involvement was observed in eight patients. Thirteen patients had a negative serologic reaction for antibody to human T-cell leukemia virus type I (HTLV-I), and one patient was considered to be a HTLV-I carrier. Polyclonal hypergamma-globulinemia was observed in 6 patients, and 6 of the 12 patients showed elevated IgE levels. Immunophenotyping of the involved lymph nodes revealed a preponderance of T-cells in all the patients. Eleven of these patients showed a predominance of CD4+ over CD8+ T-cells, and only one patient showed a predominance of CD8+ over CD4+ T-cells. Two of five patients whose gene analysis was carried out showed clonal rearrangement of the T-cell receptor beta chain gene without rearrangement of the immunoglobulin heavy chain genes. Twelve patients received doxorubicin-containing combination chemotherapy; of these, 7 patients achieved complete response, and the other 5 had partial response. Nine patients are still alive with a median follow-up period of 21 months, and five patients died during the follow-up period. Progression to high-grade T-cell lymphoma with systemic infiltration was ascertained in two of three cases for which autopsy was performed. From our experience, we recommend doxorubicin-containing combination chemotherapy as initial therapy for AIL-related lesions.
authors.affiliation: Department of Medicine, Jichi Medical School, Tochigiken, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Ohsaka
authors.forename: K
authors.initials: K
authors.lastname: Saito
authors.forename: T
authors.initials: T
authors.lastname: Sakai
authors.forename: S
authors.initials: S
authors.lastname: Mori
authors.forename: Y
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Y
authors.initials: Y
authors.lastname: Amemiya
authors.forename: S
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Y
authors.initials: Y
authors.lastname: Miura
mesh_terms: D000368:Aged; D000914:Antibodies, Viral / Q000032:analysis; D015703:Antigens, CD / Q000032:analysis; D000954:Antigens, Surface / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D015368:Human T-lymphotropic virus 1 / Q000276:immunology; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D016130:Immunophenotyping; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D009367:Neoplasm Staging; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000914:Antibodies, Viral; D015703:Antigens, CD; D000954:Antigens, Surface; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D008558:Melphalan; D011241:Prednisone; D008727:Methotrexate