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.


METHODS
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 was found. The patient underwent high-dose chemotherapy with autografting of peripheral blood stem cells; however, the tumors quickly relapsed.


CONCLUSIONS
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 for skin, a short course, and poor prognosis.
Kato
N
N
Department of Dermatology and Clinical Research Institute, National Sapporo Hospital, Japan.
Yasukawa
K
K
Kimura
K
K
Sugawara
H
H
Aoyagi
S
S
Mishina
T
T
Nakata
T
T
D018801:CD2 Antigens / Q000032:analysis*; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D006801:Humans; D008223:Lymphoma / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009303:Nasopharyngeal Neoplasms / Q000276:immunology / Q000473:pathology; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology*
D018801:CD2 Antigens; D015704:CD4 Antigens; D019002:CD56 Antigen