the two main forms of lymphoma are hodgkin lymphoma and non - hodgkin lymphoma ( nhl ) . lymphoma occurs when cells of a type of lymphocyte ( the cells of the immune system are called lymphocytes ) travel to many parts of the body , including the lymph nodes , spleen , and bone marrow , and form a mass called a tumor . the body has two main types of lymphocytes that can develop into lymphomas : b lymphocytes ( b cells ) and t lymphocytes ( t cells ) . nhls account for 60% of all lymphomas ; involvement of the nasal cavity and paranasal sinuses by these tumors is uncommon . extranodal nk / t - cell lymphomas , nasal type ( enkls ) are aggressive , locally destructive , midfacial necrotizing lesions characterized by extranodal involvement , particularly the nasal / paranasal area , and represent about 75% of all nasal lymphomas , the rest being b - cell lymphomas . incidence of enkl varies considerably in different parts of the world , but it remains a rare disease since its first description in 1933 . however , the number of new , diagnosed cases per year is on the increase , due to improved knowledge of this disease . it is more common in asian and central and south american countries than it is in north america . enkl can develop in either t cells or natural killer ( nk ) cells , which attack foreign cells . enkls are subcategorized into nasal and nasal - type nk / t - cell lymphomas according to the major site of anatomic involvement . in limited cases , nk / t - cell lymphomas may predominantly occur in extranasal sites without involvement of the nasal cavity or nasopharynx . advances in tumor cell biology have led to the ability to subclassify nhls using the world health organization ( who ) classification of lymphoma . controversy still exists regarding the normal counterpart of nk cell lymphoma : whether nk cell lymphoma represents the presence of a true nk cell or merely the presence of a t cell with abnormal cell markers is under debate . in the absence of unequivocal proof of the exact lineage of nk cell lymphoma , many investigators prefer to use the term nk / t - cell lymphoma when classifying this condition . further understanding of its development and the identification of more specific cell markers of nk and t cells will likely resolve this controversy in the future . the nasal and nasal - type nk / t - cell lymphomas have distinct presentations and prognoses , and they are believed to have different pathogeneses . compared with nodal lymphomas , sinonasal tract lymphomas are difficult to identify correctly because of the biopsy size . nasal nk / t - cell lymphomas are typically seen in the nasal and paranasal sinus areas and are associated with epstein - barr virus ( ebv ) infection . the patients often experience localized stage i / ii disease but an aggressive clinical course . radiotherapy alone has been used for the treatment of limited - stage extranodal t - cell lymphoma , but the 5-year overall survival rate is approximately 50% . recently , several groups have treated patients with irradiation of more than 40 - 50 gy followed by courses of chemotherapy , and the reported overall 5-year survival rate of this procedure reached 70% . a 25-year old illiterate female patient presented at the ear , nose , and throat ( ent ) department of the indira gandhi institute of medical science ( igims ) , patna with a complaint of gradually progressive ulcerative lesion on the infra - alar region of the right side of the face over the last 2 months . she had consulted with a local doctor and received 9 months of antitubercular treatment as advised by him / her . the lesion was increasing in size , so she came to igims for a second opinion . on examination , an ulcerative lesion was noted on the upper lip region toward the right side just below the nasal area , 4.5  3 cm and edematous margins , with yellowish dry slough on the base of the ulcer . the periculer edema was noted to include the right ala of the nose and right side of the cheek , extending up to the infraorbital region and also involving the lower lid . the chest x - ray , ultrasonography ( usg ) abdomen , and routine blood investigation were normal . the biopsy was performed by an ent surgeon and the tissue was sent for histopathological examination . microscopic examination showed the features of t - cell lymphoma , and the use of immunohistochemical ( ihc ) markers was advised . after ihc analysis , cd45 ( leukocyte common antigen or lca ) was positive ( 3 + ) [ figure 1 ] and cd3 was positive ( 1 + ) [ figure 2 ] ; the impression was of nhl t - cell immunophenotype on examination . although nk marker studies could not be done , the biological behavior of nk - positive and t - positive nasal extranodal lymphomas is similar , hence they are collectively termed nk / t - cell lymphomas . thus , based on the clinical picture , histopathology and ihc were used toward the diagnosis of enkl . immunohistochemistry cd45 positive ( 3 + ) immunohistochemistry cd3 positive ( 1 + ) after the histopathology and ihc marker reports , the patient was sent to the oncology department for further treatment . at our department , she received chemotherapy in the form of chop regimen [ cyclophosphamide 750 mg / m , hydroxydaunorubicin or doxorubicin 50 mg / m , oncovin ( vincristine ) 1.4 mg / m , and prednisone 40 mg / m ] d1-d5 , with all premedication cycles to be reported every 21-days regimen . she responded well to the chemotherapy and the ulcer has been healed completely , without any major side effects but with residual dry scar tissue . in 1982 , ishii et al . first recognized the presence of tumor cells expressing cd3 in this type of lesion and termed this disease diagnosis of these lymphomas is dependent on a series of laboratory studies , particularly cultures , but frequently a biopsy is required to establish a diagnosis . it is essential that biopsy tissue be of sufficient size and adequate technical quality in order to allow the identification of atypical cells . even though an angiocentric t - cell lymphoma often does not have the classic histopathological features of a lymphoma microscopically , it behaves in a malignant fashion and responds to the same treatment to which a lymphoma responds . it is more prevalent among asians , where it constitutes as much as 3% of nhls , and among the indigenous peoples of mexico , central america , and south america . patients with early - stage lymphoma typically experience an indolent course of disease , the tumor being restricted to the original site , but others with the disease in advanced stages suffer rapid progression to systemic dissemination often accompanied by hemophagocytosis or disseminated intravascular coagulation . extranodal nasal - type t - cell lymphomas demonstrate a predilection for the nasopharynx , palate , skin , soft tissues , orbit , gastrointestinal tract ( git ) , and testes ; lymphomas that manifest outside of the nose have a strong association with ebv in asian patients , but this strong association is not present in white patients . because this type of lymphoma occurs in organs or tissues other than the lymph nodes , it is called  extranodal . it can cause swelling of the face , discharge from nose , nasal bleeding , and nasal blockage . it is now generally accepted that a large number of biopsies must be performed to confirm the diagnosis of enkl . the histological features of nk and t - cell lymphomas are similar regardless of the site of the lesion , both consisting of sheets of atypical small , medium - sized , large , or giant sternberg - like cells . the prognosis for people with this type of lymphoma is often poor and the risk of relapse is high . people with enkls confined to the nose or nasal passages have better prognosis than those people with more widespread disease . extranodal lymphomas can also affect other organs or tissues besides the nose and may be referred to as the extranasal type . it can affect the skin , testes , soft tissues , kidneys , brain , git , and respiratory system . this entity has also been referred to as lethal midline granuloma , polymorphic reticulosis , angiocentric immunoproliferative lesion , and angiocentric t - cell lymphoma . it is characterized by vascular damage and destruction , prominent necrosis , a cytotoxic phenotype , and an association with ebv . nasal nk / t - cell lymphomas in asians follow an aggressive course , with death occurring due to local relapse or systemic spread in 50% of the cases . multidrug chemotherapy ( chop regimen ) followed by involved - field radiotherapy appears to be the most effective treatment approach . the delay in presentation and thus delay in diagnosis is possibly one of the reasons our patient appeared at an advanced stage and can be saved only with aggressive chemotherapy . this case illustrates the unusual presentation of a rare lymphoma that has responded well to chemotherapy , and the patient has been on follow - up for the past 2 years . nonhealing ulcer at an unusual site often predates the appearance of mucosal involvement and tissue necrosis by 1 year or more . , the clinician should consider extranodal lymphoma , which is a rare disease and can present with the difficulties of obtaining histological diagnosis , despite apparently adequate biopsies , because of illiteracy and ignorance about the disease , treatment , and prognosis . usually , the patient presents at an advanced stage of disease and has worse prognosis .
extranodal nk / t - cell lymphoma , nasal type ( enkl ) is a rare lymphoid neoplasm that in the past has been grouped with a variety of granulomatous diseases . 
 enkl occurs in all age groups . 
 however , it seems to occur more often in people in their 50s and affects more men than women . 
 it is strongly linked to the epstein - barr virus ( ebv ) , especially in people of asian countries . 
 because this type of lymphoma occurs in organs or tissues other than lymph nodes , it is called enkl . 
 this is a case report of a 25-year - old female patient who presented with a nonhealing ulcer at the right nasal ala involving the upper lip and cheek for the last 2 months , which had been treated with antitubercular treatment without success . after biopsy and immunohistochemical ( ihc ) analysis , 
 the patient had been diagnosed a case of extranodal t - cell lymphoma .