CD2- CD4+ CD56+ hematodermic/hematolymphoid malignancy.

Abstract:

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 was 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 for skin, a short course, and poor prognosis.

journal_name

J Am Acad Dermatol

authors

Kato N,Yasukawa K,Kimura K,Sugawara H,Aoyagi S,Mishina T,Nakata T

doi

10.1067/mjd.2001.110897

subject

Has Abstract

pub_date

2001-02-01 00:00:00

pages

231-8

issue

2

eissn

0190-9622

issn

1097-6787

pii

S0190-9622(01)58791-5

journal_volume

44

pub_type

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