Diagnostic challenges of adult T-cell leukemia/lymphoma in North America - a clinical, histological, and immunophenotypic correlation with a workflow proposal

Leuk Lymphoma. 2018 May;59(5):1188-1194. doi: 10.1080/10428194.2017.1365862. Epub 2017 Aug 25.

Abstract

Adult T-cell leukemia-lymphoma (ATLL) is caused by human T-cell lymphotropic virus type 1 (HTLV-1) and little is known about ATLL endemic to the Caribbean basin and Latin America, designated as western ATLL (W-ATLL). Due to extensive systemic involvement and nonspecific clinical presentation, the initial diagnosis in this cohort can be very challenging. We have diagnosed 60 patients with W-ATLL over a 14-year period. ATLL involves the peripheral blood, bone marrow, and cerebrospinal fluid (CSF) in 98, 87, and 52% cases, respectively; while lymphadenopathy, pulmonary infiltrates, splenomegaly, and hepatomegaly was present in 90, 82, 48, and 45% patients, respectively. While 87% patients developed hypercalcemia only 28% had lytic bone lesions. We propose that any diagnosis of a peripheral T-lymphoproliferative disorder should result in a comprehensive review of the patient's endemic, laboratory information, HTLV-1 testing for proper diagnosis, and identification due to the varied manifestations of this disease.

Keywords: HTLV-1; western ATLL; hypercalcemia.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers / metabolism*
  • Diagnostic Imaging / methods*
  • Female
  • Flow Cytometry / methods*
  • Follow-Up Studies
  • Humans
  • Immunophenotyping / methods*
  • Leukemia-Lymphoma, Adult T-Cell / diagnosis*
  • Leukemia-Lymphoma, Adult T-Cell / diagnostic imaging
  • Leukemia-Lymphoma, Adult T-Cell / immunology
  • Leukemia-Lymphoma, Adult T-Cell / metabolism
  • Male
  • Middle Aged
  • Prognosis
  • Workflow

Substances

  • Biomarkers