Clinicopathologic Features of Adult T-cell Leukemias/Lymphomas at a North American Tertiary Care Medical Center

Abstract

Human T-cell lymphotropic virus type 1 is associated with adult T-cell leukemia/lymphoma (ATLL). Published series of ATLLs seen at a United States medical institution are rare. We present the features of 4 ATLLs diagnosed at our North American tertiary care medical center from 1990 to 2012. Despite the absence of a history of origin from an endemic region, all our ATLLs demonstrated evidence of human T-cell lymphotropic virus type 1 infection. Central nervous system (CNS) involvement by ATLL was uncommon in our series, and represented only 1.6% (1/64) of all CNS B-cell or T-cell lymphomas diagnosed over a 20+ year period at our institution. Review of the medical literature reveals that the majority of CNS-involved ATLLs present with the lymphoma or acute subtype, and complete remission is difficult to achieve in these cases. CNS involvement frequently occurs with a systemic disease, which carries an aggressive clinical course with poor prognosis. In addition, CNS involvement by ATLL can be the initial presentation or seen with relapsed disease, can be the only site or be associated with other tissue sites of involvement, and may manifest with variable clinical signs/symptoms. Our retrospective study reveals that ATLLs are rare mature T-cell lymphomas in a native North American population, but the clinical and histopathologic features of ATLLs from this nonendemic region are similar to those seen from other endemic regions. Early recognition of these rare ATLLs involving uncommon sites, such as the CNS, will help optimize treatment for these infrequent mature T-cell lymphomas.

Case Report: Primary Indolent Epstein-Barr Virus-Positive T-Cell Lymphoproliferative Disease Involving the Central Nervous System

“…Although cerebral T-cell lymphomas are frequently reported ( 8 , 13 ), there are no reports on T-LPD in the CNS. This case was clinically characterized by EBV+ T-cell proliferation in the CNS, with an indolent period and developed a progressive course soon.…”

Cytomorphologic and Flow Cytometric Analysis of Cerebrospinal Fluid with T-Cell Lymphoma Involvement: A Retrospective Study of Rare Cases

“…Hsi et al [21] found 64 cases of primary or secondary CNS lymphomas at their institution in a 13-year retrospective analysis. Only 12.5% (8 cases) represented T-cell lymphomas (1 case of adult T-cell leukemia/lymphoma, 1 of monomorphic posttransplant lymphoproliferative disorder of T-cell type and 6 of T-cell lymphoblastic leukemia/lymphoma).…”

Diagnosis, prevention and treatment of central nervous system involvement in peripheral t-cell lymphomas

“…Some T-cell lymphoma entities, like adult T-cell leukemia-lymphoma (ATLL), peripheral T-cell lymphomas-no other specified (PTCL-NOS), ALK-positive ALCL (ALK + ALCL), and ENKTCL seem to be associated with an increased risk of CNS dissemination (Table 1), an event rarely reported in patients with angioimmunoblastic T-cell lymphoma Gurion et al, 2016;Ellin et al, 2015;Kim et al, 2020;Pro and Perini, 2010). CNS involvement occurs in 10-25% of patients with ATLL (Rubenstein et al, 2008;Gurion et al, 2016;Yi et al, 2011;Kim et al, 2010;Teshima et al, 1990), but it is very rare initial diagnosis (Ma et al, 2014); higher risk of CNS relapse is reported in the forms with disseminated disease, such as lymphomatous and acute forms, especially the former, which represents almost half the ATLL patients (Kitajima et al, 2002;Hsi et al, 2014). However, the rate of CNS dissemination in ATLL patients may be underestimated; this is suggested by a study showing that 14 % of ATLL patients without neurological symptoms had evidence of CNS involvement on autopsy (Teshima et al, 1990).…”

“…However, the rate of CNS dissemination in ATLL patients may be underestimated; this is suggested by a study showing that 14 % of ATLL patients without neurological symptoms had evidence of CNS involvement on autopsy (Teshima et al, 1990). In addition, CNS relapse seems to be more common in ATLL patients with leukocytosis, hypercalcemia, or elevated serum lactate dehydrogenase (LDH) levels (Hsi et al, 2014). In ENKTCL, nasal type, CNS dissemination was strongly related to the natural killer prognostic index (NKPI includes B symptoms, stage III or IV, elevated serum LDH concentration, and lymph node involvement), with a CNS relapse rate of 1.8 % for patients with NKPI score 1-2 and 10.2 % for patients with NKPI 3-4 (Kim et al, 2010).…”