Blood Res 2018; 53(1): 8  https://doi.org/10.5045/br.2018.53.1.8
A weeping ulcer that vanished with a ‘SMILE’
Ankur Jain1, Gaurav Prakash1*, Amanjit Bal2, Pankaj Malhotra1, and Subhash Varma1

1Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

2Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Correspondence to: Gaurav Prakash, M.D., Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. drgp04@gmail.com
Received: May 19, 2017; Revised: May 22, 2017; Accepted: June 25, 2017; Published online: March 31, 2018.
© The Korean Journal of Hematology. All rights reserved.

 

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A 30-year-old man of Indian origin complained of a two-month-old ulcer on his face that remained unhealed. He denied any nasal obstruction, epistaxis, headache, or fever. A painless crusted lesion (5×5 cm) with surrounding induration localized over the right side of the nose along with periorbital edema (A) was observed. There was no pallor, palatal lesion, nasal mass, lymphadenopathy, or hepato-splenomegaly. A punch biopsy from the edge of the lesion was suggestive of extranodal natural killer (NK)/T-cell non-Hodgkin's lymphoma (NK/T-NHL), nasal type. Immunohistochemistry revealed positivity for cytoplasmic cluster of differentiation (CD) 3 and 56, granzyme B, and Epstein-Barr virus (EBV)-encoded-RNA by in-situ hybridization (EBER-ISH) thereby confirming the diagnosis of NK/T-NHL, nasal type (C, D). Peripheral blood analysis by polymerase chain reaction revealed 1,200 copies/mL of EBV-DNA. PET-CT scan, and MRI of orbit localized the disease to skin and subcutaneous tissue of the face (extranasal), with no periorbital extension. Blood investigations revealed a normal complete blood count (CBC), renal and liver function tests, and normal lactate levels. Treatment with chemotherapy (SMILE protocol: Steroids, Methotrexate, Ifosphamide, L-asparaginase, and Etoposide) and sandwiched radiotherapy led to complete resolution of the disease, as assessed by a repeat PET-CT scan (B). Extranodal NK/T cell lymphomas, nasal type, are highly aggressive neoplasms of the upper aerodigestive tract, constituting about 5–15% of NHL in Asian countries. ‘Isolated’ cutaneous involvement in NK/T NHL, nasal type, is rare, mandating its awareness amongst hematologists.



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