CD30 NEGATIVE/CD4 POSITIVE CYTOTOXIC T CELL LYMPHOMA: A RARE CUTANEOUS LYMPHOPROLIFERATIVE DISORDER

Abstract

Introduction: There are few reports of primary cutaneous lymphomas with CD4 + / CD30 negative cytotoxic T cell immunophenotype. 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. We report the clinical characteristics, immunophenotype and outcome of a patient with this rare type of T cell proliferation. Case report: A 66 year-old male presented a two years history of widespread erythematous scaly rash and nodules on the skin of the chest, which biopsy showed an atypical lymphocytic infiltrate in the dermis with epidermotropism. Immunohistochemical staining showed positivity for CD2, CD3, CD4, CD7, and no expressions of CD8 and CD30. The histopathological conclusion was Mycosis Fungoides (MF). Bone marrow (BM) biopsy was normal at diagnosis. Peripheral blood immunophenotyping detected 88 small T cells per mL with weak expressions of CD2, CD3 and CD5, normal expressions of CD4, CD27, CD28, CD45RA and partial expressions of CD7, CD25, CD26, CD45RO, CD56. Therefore, this phenotype was not characteristic of Sezary cells. Despite successive treatments (PUVA plus corticosteroids, methotrexate plus interferon, CHOP), the patient developed a progressive disease with worsening of skin lesions, disseminated lymphadenopathy, fever, night sweats. Inguinal lymph node biopsy was compatible with large cell T cell lymphoma, with immunostaining positivity for CD3 and CD4 while CD8, CD 30 and ALK were negative. The BM immunophenotype revealed large T cells with expressions of CD2, CD3, CD4, CD11c, CD26, CD27, CD38, CD45, CD45RO, CD56, CD94, CD197, cyGranzyme and cyPerforin with monoclonal pattern by the expression of T-cell receptor β1 constant region (TRBC1). These cells did not express CD5, CD7, CD16, CD25, CD28, CD30, CD57, CD45RA, cyTCL1. Discussion and conclusion: The immunophenotype observed does not meet the criteria for MF diagnosis. The immunophenotype observed was compatible with monoclonal cytotoxic CD4 cells. The clinical and immunophenotypic features suggested that diagnosis of a rare case of CD30 negative Anaplastic Large Cell Lymphoma (ALCL) transformed from a small cell variant of ALCL, with an aggressive clinical course. The presence of cytotoxicity markers such as cyPerforin and cyGranzyme are related to greater cytolytic activity, which correlates with more aggressive clinical behavior.