CD56+ Lymphoma With Skin Involvement

Abstract

Background: Extranodal lymphomas expressing CD56 (neuronal cell adhesion molecule) are characterized by a high incidence of cutaneous involvement and a very aggressive clinical course. Knowledge about the prognosis and clinicopathologic features of CD56 + lymphomas with skin involvement is very limited.Objectives: To determine survival and prognostic factors for extranodal CD56 + lymphomas with skin involvement and to describe their clinicopathologic features.Design: Retrospective literature survey and case studies.Patients: A total of 181 patients with CD56 + lymphoma involving the skin: 177 cases from the literature and 4 new cases.Main Outcome Measure: Survival and its dependence on the following putative prognostic factors: staging, histopathologic findings, lymphocyte markers, Tcell receptor gene rearrangement, Epstein-Barr virus infection, treatment modality.Results: Three major subtypes of CD56 + lymphoma in the skin were distinguished: blastic lymphoma, nasal-type natural killer-cell/T-cell lymphoma, and subcutaneous panniculitislike lymphoma. The disease disseminated readily, mainly to lymph nodes, bone marrow, the central nervous system, and the liver, but 45% of patients had a purely cutaneous disease at presentation. All subtypes had a very aggressive course with a median survival of 14 months. The main risk factors were age older than 55 years (hazard ratio [HR], 2.5; 95% confidence interval [CI], 1.8-3.2), systemic dissemination at presentation (HR, 2.0; 95% CI, 1.5-3.3), and lack of CD30 (HR, 3.8; 95% CI, 1.4-4.9) or CD4 expression (HR, 1.56; 95% CI, 1.06-2.57). The different treatment modalities did not improve survival.Conclusions: CD56 + lymphomas involving the skin are rare and extremely aggressive regardless of their histologic presentation and the extent of skin involvement. No effective treatment is available. The risk of death is particularly increased in older patients with CD30 − CD4 − lymphomas.

A Case of Primary Cutaneous Nasal Type NK/T-Cell Lymphoma and Review of the Literature

“…Detection of the Epstein-Barr virus genome by in situ hybridization [7] or polymerase chain reaction is an essential argument for the diagnosis of NK nasal type lymphoma. Our patient also had a TCRγ major monoclonal rearrangement in the cutaneous tumor, a result found in rare γ/δ T-cell lymphomas [2] and in cytotoxic T lymphomas with a NK-like phenotype [9,10,11,12]. However, CD4 negativity, the detection of EBV sequences and the germline configuration of TCRδ are consistent with the diagnosis of NKTL-NT.…”

Functions and Uses of Immunohistochemical Stains in Cutaneous Infiltrates of Hematopoietic Origin: A Review for the Practicing Dermatologist

“…80,81 Although most CD56-positive cutaneous lymphomas have an overall poor prognosis, CD30-positive cutaneous ALCL maintains a good prognosis even with CD56 positivity. 82 As mentioned previously, malignant plasma cells may also express CD56. When part of multiple myeloma, CD56 positivity indicates a higher incidence of lytic bone lesions and an overall better prognosis than CD56-negative myeloma.…”

Immunocytochemistry and the diagnosis of cutaneous lymphoma

“…For example, Gniadecki et al. 67 identified three types of CD56+ lymphoma in the skin; ‘blastic’, nasal type NK/T and SPTCL. Harvell et al.…”