Journal Information
Vol. 42. Issue S2.
Pages 240-241 (November 2020)
Download PDF
More article options
Vol. 42. Issue S2.
Pages 240-241 (November 2020)
DOI: 10.1016/j.htct.2020.10.403
Open Access
L. Pereira, L.G.R. Barbosa, J.E. Conti-Spilari, C.M. Bertolucci, A.R. Severino, R. Teotonio, M. Higashi, E.R. Mattos, M.R.V. Ikoma-Colturato
Hospital Amaral Carvalho, Jaú, SP, Brazil
Article information
Full Text

Introduction: Primary CD30 negative cutaneous T-lymphomas are rare and correspond to less than 5% of all cutaneous T-lymphomas. Cytotoxic CD4+ T cell lymphomas are also uncommon. Here, we report a rare case of primary cutaneous lymphoma of CD4 +/CD30 negative cytotoxic T cells. Case report: A 66-year-old male was referred to our service with a two years history of widespread erythematous scaly and itchy rash reaching 2/3 of body skin surface and 3 nodules on the skin of the chest, that had no improvement with topical measures. Skin biopsy showed an atypical lymphocytic infiltrate in the dermis with epidermotropism. Immunohistochemical staining showed: CD2+, CD3+, CD4+, CD7+, CD8 and CD30 negative. Bone marrow (BM) biopsy was normalThe diagnosis was mycosis fungoides (MF) in clinical stage IIIA, since the extent of the erythroderma was greater than 80% of the body surface. Peripheral blood (PB) immunophenotyping detected 88 small T cells/mL, with dim expressions of CD2, CD3 and CD5, normal expressions of CD4, CD27, CD28, CD45RA, and partial expressions of CD7, CD25, CD26, CD45RO, CD56, that does not correspond to a Sezary cell immunophenotype. Initially, he was treated with PUVA plus corticosteroids with no response. Sequentially, he underwent chemotherapy with methotrexate plus interferon. Although the skin lesions improved, 4 months later he developed a bilateral enlargement of the inguinal lymph nodes. Inguinal lymph node biopsy was compatible with large T cell lymphoma, CD3 +, CD 4+ while CD8, ALK and CD 30 were not expressed. He received 6 cycles of CHOP regime. After the 3rd cycle, PET-CT showed lesions without residual uptake (Deauvile I scale). After 6 cycles, he presented progressive disease with fever and night sweats, with disseminated axillary, inguinal, retroperitoneal lymphadenopathy and bone lesions, confirmed by PET-CT (Deauville 5). The BM immunophenotype revealed large cytotoxic T cells CD2+, CD3+, CD4+, CD11c+, CD26+, CD27+, CD38+, CD45+, CD45RO+, CD56+, CD94+, CD197+, Granzyme+ and Perforin+, with monoclonal pattern expression of TRBC1, without expressions of CD5, CD7, CD16, CD25, CD28, CD30, CD57, CD45RA, cyTCL1. Discussion: The PB and BM immunophenotypes did not meet the criteria for the diagnosis of MF. The immunophenotype observed was compatible with monoclonal cytotoxic CD4 cells, which may correspond to a rare case of CD30 negative Anaplastic Large Cell Lymphoma transformed from a small cell variant of anaplastic large cell lymphoma, which has an aggressive clinical course. Based on the few reported cases, it can be suggested that the prognosis is the same as that seen in other forms of cutaneous T lymphomas, in which the predominance of small cells has an indolent clinical course, whereas large T cell lymphomas are expected have a more aggressive clinical course. But it is irrefutable that the presence of markers such as Perforin and Granzyme are related to greater cytolytic activity, which correlates with more aggressive behavior. Therapeutic approaches for these patients range from topical treatments in the early stages, or intensive chemotherapy and stem cell transplantation for more aggressive diseases. But the treatments available for these T-cell lymphomas are not effective enough and new treatment modalities are needed.

Hematology, Transfusion and Cell Therapy

Subscribe to our newsletter

Article options