IADVL
Indexed with PubMed and Science Citation Index (E) 
 
Users online: 1835 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
  Search
 
  
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
   Article in PDF (893 KB)
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

 
  In this article
   References
   Article Figures

 Article Access Statistics
    Viewed1450    
    Printed25    
    Emailed0    
    PDF Downloaded45    
    Comments [Add]    

Recommend this journal

 


 
 Table of Contents    
LETTER TO THE EDITOR
Year : 2014  |  Volume : 80  |  Issue : 6  |  Page : 564-566

NK/T-cell lymphoma nasal type with an unusual clinical course


1 Department of Dentistry, Faculty of Medicine, University Hospital in Hradec Králové, Charles University in, Czech Republic
2 4th Department of Medicine-Hematology, Faculty of Medicine, University Hospital in Hradec Králové, Charles University in, Czech Republic
3 Fingerland Department of Pathology, Faculty of Medicine, University Hospital in Hradec Králové, Charles University in, Czech Republic

Date of Web Publication7-Nov-2014

Correspondence Address:
Vladimira Radochova
Sokolská 581, 50005 Hradec Králové
Czech Republic
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.144213

Rights and Permissions



How to cite this article:
Radochova V, Radocha J, Nova M, Belada D, Slezak R. NK/T-cell lymphoma nasal type with an unusual clinical course . Indian J Dermatol Venereol Leprol 2014;80:564-6

How to cite this URL:
Radochova V, Radocha J, Nova M, Belada D, Slezak R. NK/T-cell lymphoma nasal type with an unusual clinical course . Indian J Dermatol Venereol Leprol [serial online] 2014 [cited 2020 Feb 22];80:564-6. Available from: http://www.ijdvl.com/text.asp?2014/80/6/564/144213


Sir,

Non-Hodgkin's lymphomas present at an extranodal site in up to 40% of the patients. Extranodal nasal natural killer (NK)/T-cell lymphoma which represents about 1.4% of all lymphomas is more prevalent in East Asia and in certain parts of Central and South America and is frequently associated with Epstein-Barr virus (EBV) infection (60-90%). Most cases express CD56 (around 80%), CD2, granzyme B, and T-cell-restricted intracellular antigen-1 (more than 90%) and also carry p53 deletion. Systemic dissemination occurs later in the course of the disease. Patients with NK/T-cell lymphoma have a probability of survival at 5 years ranging from 37.9% to 43.5%. [1] We describe a patient with an unusual initial presentation and an unusual course during relapse.

A 56-year-old white man was referred for consultation regarding a lip swelling and non-healing wound after tooth extraction. One month prior to presentation, the patient underwent extraction of tooth 23 for the diagnosis of chronic periodontitis. However, soon afterwards, the patient developed a painless swelling of the lip, but he complained of mild pain during food intake in the area around the tooth extraction wound. He was treated with antihistaminic drugs without much relief. Almost simultaneously, erosions appeared on the mucosa around the extraction wound and on the palate. These lesions were sensitive after food intake. The patient denied any history of fever, weight loss, or nasal stuffiness. In addition, he had a history of arterial hypertension and had a 15-pack year history of smoking cigarettes. Examination revealed a swelling of the upper lip, which was soft and painless. The lymph nodes in the submandibular area were enlarged. Intraoral examination revealed a deep necrotic ulcer with a ragged border, measuring 20 mm in diameter, located on the left side of the hard palate. An erosion was present on the mucosa of the alveolar arch in the area of the wound [Figure 1].
Figure 1: Ulcer after tooth extraction upon presentation

Click here to view


Biopsy of the lesions showed ulcerated mucosa infiltrated with atypical lymphoid cells with angulated nuclei and bright cytoplasm with invasion into the surrounding tissues [Figure 2]. Immunohistochemistry showed strong positivity for leukocyte common antigen, CD2, perforin, and granzyme B. Ki-67 showed a high proliferation activity of around 60%. EBV-encoded small RNA was negative. The diagnosis of NK/T-cell lymphoma nasal type was established. Since no other localization of lymphoma was seen during the initial staging (stage I E), the patient was treated with 6 cycles of CHOEP-14 (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone) which led to partial remission. This was followed by field radiation therapy using intensity-modulated radiation therapy technique (27 × 2 Gy) which resulted in complete remission. Five months later, the patient was again admitted to the pulmonary department with fever and multiple pulmonary infiltrates [Figure 3]. After the initial suspicion of pneumonia was ruled out, open lung biopsy was performed which confirmed early lung dissemination of the lymphoma. He was given salvage chemotherapy ESAP (etoposide, methylprednisolone, cytosine arabinoside, and cisplatin). However, despite therapy, the patient's condition worsened, and he died 14 days later.
Figure 2: Small lymphocytic infiltrates invading the tissue (H and E, ×200)

Click here to view
Figure 3: Multiple nodular lymphoma infiltrates mimicking lung infection

Click here to view


NK/T-cell lymphoma, nasal type has a very aggressive clinical course and a poor outcome. Involvement of the lung has been described only twice. [2],[3] We would like to stress this possible clinical feature of NK/T-cell lymphoma. Invasive diagnostic workup including biopsy of the lung is warranted in such cases in order to avoid misdiagnosis and delay in treatment. Palatal ulcers and non-healing ulcers in the oral cavity could represent a potentially malignant lesion which should warrant an immediate biopsy. It is frequently necessary to perform repeated examination before the diagnosis is made. In our patient, the palatal ulcer was first treated for suspected allergy and then as a wound after tooth extraction before he was referred to a specialized unit and biopsy was performed. In our case, CD56 was negative, but perforin and granzyme B were positive thus leading to the diagnosis. Current data suggest that many B-cell and some T-cell malignancies are associated with the presence of EBV in the tissue but this was not detected in our patient. According to a recent study, positron emission tomography/computerized tomography (PET/CT) is the preferred technique for imaging this type of lymphoma as it has significantly better sensitivity than conventional CT (97.7% vs. 80.7%, P < 0.001) and the findings can also affect the treatment plan and radiotherapy planning in more than 40% of patients. [4] Treatment consists of conventional CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or similar regimens followed by radiation therapy. Newer approaches may include asparaginase, gemcitabine or autologous stem cell transplantation. [5] The prognosis of this lymphoma remains poor. The 5-year disease free survival of nasal lymphoma is about 25% and the 2-year disease free survival in patients with extranasal lymphoma is about 10%.

 
  References Top

1.
Al-Hakeem DA, Fedele S, Carlos R, Porter S. Extranodal NK/T-cell lymphoma, nasal type. Oral Oncol 2007;43:4-14.  Back to cited text no. 1
    
2.
Morovic A, Aurer I, Dotlic S, Weisenburger DD, Nola M. NK cell lymphoma, nasal type, with massive lung involvement: A case report. J Hematop 2010;3:19-22.  Back to cited text no. 2
[PUBMED]    
3.
Laohaburanakit P, Hardin KA. NK/T cell lymphoma of the lung: A case report and review of literature. Thora×2006;61:267-70.  Back to cited text no. 3
    
4.
Moon SH, Cho SK, Kim WS, Kim SJ, Chan Ahn Y, Choe YS, et al. The role of 18F-FDG PET/CT for initial staging of nasal type natural killer/T-cell lymphoma: A comparison with conventional staging methods. J Nucl Med 2013;54:1039-44.  Back to cited text no. 4
    
5.
Yamaguchi M, Kwong YL, Kim WS, Maeda Y, Hashimoto C, Suh C, et al. Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: The NK-Cell Tumor Study Group study. J Clin Oncol 2011;29:4410-6.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article

    

Online since 15th March '04
Published by Wolters Kluwer - Medknow