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Extranodal natural killer/T-cell lymphoma, nasal-type.
Dimitrios Chorianopoulos1, Konstantinos Samitas, Stylianos Vittorakis
1Department of Pulmonary Medicine, Athens Medical School, Sotiria General Hospital, Athens, Greece. dchorian@gmail.com
Skinmed
|September 16, 2010
Summary
This case study details a rare extranodal extranasal natural killer/T-cell lymphoma, CD56-positive, Epstein-Barr virus-negative, and TCR-negative. The patient presented with skin lesions and pulmonary infiltrates, ultimately succumbing to CNS involvement.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Extranodal natural killer/T-cell lymphoma (ENKTL), nasal-type, is an aggressive lymphoid malignancy.
- Diagnosis can be challenging, particularly in extranasal presentations without initial sinonasal involvement.
Observation:
- A 51-year-old man presented with constitutional symptoms, dry cough, fever, and ulcerated skin lesions.
- Initial investigations revealed pulmonary infiltrates and a nasal mass.
- Skin biopsy showed lymphoproliferative infiltration consistent with ENKTL.
Findings:
- Immunohistochemistry confirmed CD56 positivity and cytoplasmic CD3 expression.
- Epstein-Barr virus (EBV) and T-cell receptor (TCR) gene rearrangements were negative.
- The final diagnosis was extranodal extranasal ENKTL, CD56-positive, EBV-negative, TCR-negative.
Implications:
- This case highlights the importance of considering ENKTL in the differential diagnosis of unexplained skin lesions and systemic symptoms.
- The diagnostic challenges of extranasal ENKTL underscore the need for comprehensive histopathological and molecular analysis.
- Despite initial treatment response, the patient experienced rapid progression and central nervous system involvement, emphasizing the aggressive nature of this lymphoma.
