Hodgkin-like adult T-cell leukemia/lymphoma that developed during the follow-up of HTLV-1 associated myelopathy/tropical spastic paraparesis

Abstract

Hodgkin-like adult T-cell leukemia/lymphoma (ATLL) is a rare variant of ATLL, which represents the early neoplastic phase of ATLL that follows an indolent clinical course compared with typical ATLL. Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a neurological disorder characterized by the paralysis of lower limbs and urinary disturbance. Although these diseases are caused by HTLV-1 infection, there are no reports describing the coexistence of Hodgkin-like ATLL and HAM/TSP. Here, we report the first case of Hodgkin-like ATLL complicated by HAM/TSP. The patient was a 56-year-old man with right inguinal lymphadenopathy who had been using the neurology outpatient service for 13 years after being diagnosed with HAM/TSP. He was unable to receive intensive chemotherapy or allogeneic stem cell transplantation due to a poor performance status, but his condition was stable for approximately two years.

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.…”