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Published on: December 29, 2012
Lymphomatoid papulosis with a natural killer-cell phenotype
M W Bekkenk1, P M Kluin, P M Jansen
1Department of Dermatology, Leiden University Medical Centre, Albinusdreef 2, 2300 RC Leiden, the Netherlands. m.w.bekkenk@lumc.nl
The British Journal of Dermatology
|September 5, 2001
Summary
Lymphomatoid papulosis (LyP) typically involves T-cells, but this study details a rare case with natural killer (NK) cells. Despite the NK cell phenotype, the patient showed a favorable prognosis and treatment response, similar to typical LyP.
Area of Science:
- Dermatology
- Hematology
- Immunology
Background:
- Lymphomatoid papulosis (LyP) is a rare skin condition characterized by recurrent, self-healing papulonodular eruptions.
- Histologically, LyP exhibits features of a CD30-positive cutaneous T-cell lymphoma, with atypical T-cells typically expressing CD3, CD4, and CD30 markers.
Observation:
- This report details an atypical case of LyP with a distinct cellular phenotype.
- The atypical cells in this patient expressed a CD3-, CD4-, CD8-, CD30+, CD56+ profile.
- Phenotypic and genotypic analyses confirmed these cells possessed a Natural Killer (NK)-cell phenotype.
Findings:
- NK-cell lymphomas generally carry a poor prognosis.
- Despite the NK-cell phenotype, the patient's condition demonstrated a prolonged course (over 20 years) with waxing and waning lesions.
- The patient responded exceptionally well to low-dose oral methotrexate therapy.
Implications:
- This case challenges the typical understanding of LyP cellular origins.
- The findings suggest that LyP with an NK-cell phenotype may exhibit a clinical course and treatment response similar to T-cell LyP.
- Further research is warranted to understand the implications of NK-cell involvement in LyP and its therapeutic strategies.
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