Characterization of the spectrum of postthymic T-cell malignancies in Taiwan. A clinicopathologic study of HTLV-1-positive and HTLV-1-negative cases

Cancer. 1988 May 15;61(10):2060-70. doi: 10.1002/1097-0142(19880515)61:10<2060::aid-cncr2820611022>3.0.co;2-d.

Abstract

Postthymic T-cell malignancy shows marked geographic, clinicopathologic, and prognostic diversity. The frequency and spectrum of T-cell malignancies in Taiwan were investigated. Fifty-two patients (35 male and 17 female) with a median age of 49 years, were consecutively encountered between October 1983 and April 1987; these accounted for 39% of non-Hodgkin's lymphoma cases seen in our institutions. Ten patients (19.3%) had adult T-cell leukemia/lymphoma (ATL) associated with human T-cell leukemia virus (HTLV-1). Patients with ATL had disease similar to that reported from southwestern Japan and the Caribbean. They had frequent skin lesions (60%), hypercalcemia (40%), and a rapid clinical course with a median survival of 1.3 years. The 35 HTLV-1-negative peripheral T-cell lymphomas (PTL) were similar to PTL in western countries, manifesting frequent visceral, cutaneous, and vascular tropisms. Marrow involvement was documented at presentation in 39% and Stage III/IV disease in 80% of the PTL patients. The histology of PTL usually expressed prominent reactive features which is distinct from that in ATL. Several subcategories could be defined: Hodgkin's-like PTL in nine patients, T-zone lymphoma in three, angioimmunoblastic lymphadenopathy-like lymphoma in one, Lennert's lymphoma in three, and angioinvasive lymphoma in four. Two HTLV-1-negative PTL had neoplastic cells with clover-shaped nuclei and were designated as ATL-like. Morphologic classification based on the modified Working Formulation showed prognostic correlation, with median survival of less than 6 months for large cell/immunoblastic PTL, compared with 5 years for patients with small/medium cell PTL. Both low- and high-grade PTL seem to represent an incurable disease. Classical cutaneous T-cell lymphoma (seven cases) is relatively unusual in Taiwan, compared with the frequency of PTL. Post-thymic T-cell malignancies in Taiwan include HTLV-1-positive and HTLV-1-negative diseases, both of which have a poor prognosis and resemble similar T-cell malignancies in the East and West.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Antigens, Neoplasm / analysis
  • Antigens, Viral / analysis
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Deltaretrovirus / immunology
  • Deltaretrovirus Infections / drug therapy
  • Deltaretrovirus Infections / epidemiology
  • Deltaretrovirus Infections / pathology*
  • Female
  • Humans
  • Lymphoma, Non-Hodgkin / drug therapy
  • Lymphoma, Non-Hodgkin / epidemiology
  • Lymphoma, Non-Hodgkin / pathology*
  • Male
  • Middle Aged
  • Prognosis
  • T-Lymphocytes / pathology*
  • Taiwan

Substances

  • Antigens, Neoplasm
  • Antigens, Viral