Adult T-cell leukemia/lymphoma with Epstein-Barr virus–positive Hodgkin-like cells

Abstract

SUMMARY Hodgkin-like cells (HLC) have been described in a variety of non-Hodgkin lymphomas (NHL) including chronic lymphocytic leukemia (CLL) and peripheral T-cell lymphoma (PTCL). There have been rare reports in the Japanese population of human T-cell lymphotrophic virus-1 (HTLV-1)-associated adult T-cell leukemia/lymphoma (ATLL) harboring HLC; however, no similar cases have been described in western patients. We report a 53-year-old African-American man that presented with progressive weakness and lethargy, and was found to have generalized lymphadenopathy and hypercalcemia. A lymph node biopsy showed involvement by ATLL with scattered Epstein-Barr virus (EBV)-positive cells, some of which resembled Hodgkin cells that had a B-cell phenotype, consistent with an Epstein-Barr virus-lymphoproliferative disorder (LPD). The patient had stage 4 disease with bone marrow involvement. In light of the associated B-cell lymphoproliferative process, the patient was treated with six cycles of intensive chemotherapy that targeted both the ATLL and the EBV-LPD that resulted in a complete response. An awareness of the association of EBV-LPD with Hodgkin-like cells in the context of ATLL is necessary to avoid potential misdiagnosis and to aid in therapeutic decisions.

Peripheral T-cell Lymphomas of Follicular T-Helper Cell Derivation With Hodgkin/Reed-Sternberg Cells of B-cell Lineage

“…Two cases of adult T-cell leukemia/lymphoma (ATLL) were included, one of which was previously reported. 16 A summary of the demographic, clinical and molecular data is presented in table 2. All patients were adults, with a median age of 63 years (n=57).…”

“…This scenario also explains the development of EBV-positive HRS-like cells in ATLL, a lymphoma associated with marked immunosuppression. 16,20 However, in our review of 57 cases of T-cell lymphoma with HRS-like cells, we encountered 5 cases in which the HRS-like cells were negative for EBV. Thus, other pathogenetic mechanism must be sought.…”

“…Polymerase chain reaction (PCR) or genomic Southern Blot analysis (case 3) were carried out on whole tissue extracts from paraffin-embedded tissue. The PCR reactions were run according to the protocols previously detailed by Venkataraman et al 16 and Eberle et al 15 For the TRG locus, we used primers that interrogate all of the known Vγ family members and the J1/2, JP1/2, and JP joining segments. 17 For the IGH locus, we employed consensus primers directed against the joining region (J H ) with forward primers for the V H framework (FR) III and V H FR-II; for the IgK analysis, the primer sets interrogated rearrangements involving the Vκ loci and Jκ (tube A), the Vκ locus and the κDE locus (tube B), and the κ-intron RSS locus and the κDE locus (tube B) (InvivoScribe Technologies, Inc, San Diego, CA).…”

Thomas Hodgkin and Hodgkin lymphoma

“…AITL typically has an admixture of reactive cells, including eosinophils and plasma cells, and may mimic, in particular, MCCHL. Less commonly, peripheral T cell lymphoma, NOS [74], and rarely adult T cell leukemia/lymphoma (HTLV1+) [74,75] contain Reed-Sternberg-like B cells. Recently, cases of the follicular variant of peripheral T cell lymphoma, NOS, which has a less polymorphous composition, and may also contain EBV+ Reed-Sternberg-like B cells have been described that have been mistaken initially for LRCHL [76].…”

Adult T cell leukaemia/lymphoma in Kerala, South India: are we staring at the tip of the iceberg?

“…A study by Ohtsubo et al states that the Epstein-Barr virus (EBV) positivity in these cells most likely stems from the underlying immunodeficiency state precipitated by HTLV-1 infection. CD21 is the EBV receptor and is reported to be over-expressed in CD4+ T cells in ATLL as a result of the stimulatory influence of the HTLV-1 Tax protein; however, evidence of EBV DNA integration could not be demonstrated in these cells, questioning the role of EBV in ATLL leukemogenesis [33,34]. In contrast, a recent study by Ueda et al purports that co-infection with HTLV-1 and EBV may be associated with a greater likelihood of organ involvement and a more aggressive course through the enhanced expression of adhesion molecules via increased interleukin-4 (IL-4) signalling [33,35].…”