Prolonged lymphocytosis as the first manifestation of Hodgkin-like adult T-cell leukemia/lymphoma

Abstract

Hodgkin-like ATLL is a rare variant of adult T-cell leukemia/lymphoma (ATLL), a disease caused by human T-cell lymphotropic virus type-1 (HTLV-1). At admission, a 46-year-old female presented with lymphadenomegaly, lymphocytosis, slight elevation of LDH blood level, and acid-alcohol resistant bacilli in sputum and was being treated for pulmonary tuberculosis (Tb). She had lymphocytosis in the previous 20 months. Serology for HTLV-1 was positive. Lymph node was infiltrated by medium-sized lymphocytes with scattered Hodgkin and Reed-Sternberg-like cells CD30+, CS1-4+, and CD79a+. Background cells were CD4+ and CD25+. A clinical diagnosis of favorable chronic ATLL was given. She was treated with chemotherapy but later progressed to acute ATLL and ultimately died. Hodgkin-like ATLL should be considered in the histological differential diagnosis with Hodgkin lymphoma since treatment and prognosis of these diseases are distinct. It is also important to search for HTLV-1 infection in patients with unexplained prolonged lymphocytosis.

Clinical and cytopathological characteristics of HTLV‐1+ hodgkin lymphoma

“…1 HL-like ATLL is rare and displays scattered CD30 + and CD15 + Hodgkin and Reed Sternberg (RS) cells, often with Epstein-Barr virus (EBV) infection against a background of small-and medium-sized lymphocytes with atypical nuclear contours. [3][4][5][6] Both HL-like ATLL and the more common pleomorphic-type ATLL show integration of HTLV-1 proviral DNA and rearrangements of the T-cell receptor (TCR) Cβ1 gene in tumor tissues. The 5-year survival of patients with HL-like ATLL is 25.9%, which is greater than that of the other ATLL histological subtypes.…”