Peripheral T‐cell lymphomas, unspecified (or not otherwise specified): a review

Abstract

Peripheral T-cell lymphomas (PTCL) comprises a heterogeneous group of haematological tumours, which originate from mature T-cells, and constitute less than 15% of all non-Hodgkin's lymphomas (NHLs) in adults. The current WHO classification recognizes nine distinct clinicopathologic peripheral T-cell NHLs, being the 'unspecified variant' (PTCL-U) the most common subtype. These neoplasms often present in advanced stage at diagnosis, and most commonly have an aggressive clinical course requiring prompt treatment. The rarity of these tumours requires additional studies to better understand their biology and search for new therapies which may hopefully improve the dismal outcome of most patients. This review aims to describe the pathobiological aspects as well the clinical characteristics and current therapeutic strategies of the PTCLs, with special attention to the group of PTCL-U

Cutaneous Manifestations of Unspecified Peripheral T-Cell Lymphoma May Be Indicative of Disease Activity and Predict Response to Therapy

“…8,9 Skin involvement is a rare occurrence in PTCL-u, with reports of cutaneous lesions in 10% to 18% of patients. 1,10 Unlike primary cutaneous T-cell counterparts, cutaneous involvement in PTCL-u has been described as a poor prognostic factor, with 5 year survival rates of 12% despite treatment. 11 Cutaneous response to romidepsin therapy in our patient allowed us to predict corresponding systemic response.…”

“…Peripheral T-cell lymphoma (PTCL) comprises a group of mature T-cell malignancies that together make up less than 15% of all non-Hodgkin's lymphoma; PTCL has been reported to directly involve the skin in a minority of cases. 1 Unspecified PTCL (PTCL-u) makes up the majority of these lymphomas (60% to 70%). 2 We report a patient with PTCL-u with secondary cutaneous involvement who was treated with a new histone deacetylase inhibitor, romidepsin (Istodax; Celgene, Summit, NJ).…”

Clinicopathologic Conference: Case 2

“…The frequency depends largely on the country of origin of the patients, with a frequency of 22.6% being reported in a review of cases of peripheral T-cell and natural killer (NK)/ T-cell lymphomas from North America, Europe and Asia [3]. The WHO reports this tumor as accounting for about 50% of the peripheral T-cell lymphomas seen in Western countries [1].…”

“…Patients often have high stage disease at presentation, with involvement of multiple extranodal sites. Cases with marrow and blood involvement often overlap with aggressive NK-cell leukemia [1,3].…”

Comparison of Long-Term Clinical Outcomes of CHOP Chemotherapy between Japanese Patients with Nodal Peripheral T-Cell Lymphomas and Those with Diffuse Large B-Cell Lymphoma in the Study Group of the Tohoku Hematology Forum

“…1,2 However, their incidence shows significant geographical and racial variation, such that they are much more common in Asia, including Japan, than in North America or Europe. 3,4 The most common PTCL subtypes are peripheral T-cell lymphoma, unspecified (PTCL-u or PTCL, not otherwise specified) (25.9%), angioimmunoblastic T-cell lymphoinvolvement of extranodal tissues, including bone marrow, skin, and spleen, and a majority have advanced disease with B symptoms. 6 Moreover, PTCLs include primary extranodal Tcell lymphoma, especially cutaneous peripheral T-cell lymphomas.…”