CD2-CD4+CD56+ hematodermic/hematolymphoid malignancy

Background: CD2(-)CD4(+)CD56(+) lymphoid malignancy has been only rarely reported the last 5 years. It is characterized by a high incidence of cutaneous involvement, cytologically agranular cells, aggressive clinical course, and negative Epstein-Barr virus (EBV) involvement. Observation: We describe a Japanese patient with a unique hematolymphoid malignancy characterized by an involvement of skin, nasopharyngeal region, bone marrow, lymph node, and a CD4(+)CD43(+)CD56(+) CD2(-)CD3(-)CD8(-) and terminal deoxynucleotidyl transferase phenotype. Clinically: the cutaneous eruptions were purplish, hard, multiple nodules. Histologically a massive proliferation of atypical pleomorphic cells with medium-sized nuclei were observed throughout the dermis. No clonal rearrangement of T-cell receptor (TCR)-beta gene or immunoglobulin heavy chain J gene was found, and no positive identification of EBV by in situ hybridization for EBV-encoded small nuclear RNA aas found. The patient underwent high-dose chemotherapy with autografting of peripheral blood stem cells; however the tumors quickly relapsed. Conclusion: We gathered data from 17 cases of lymphoid malignancy from the literature sharing immunophenotypic and genotypic features similar to those of our case, including CD2(-)CD4(+)CD56(+) and germline rearrangement of TCR. Although the cellular origin could not be decided, this malignancy was found to have 100%; affinity fur skin, a short course, and poor prognosis.