non - hodgkin 's lymphomas ( nhls ) are a heterogeneous group of lympho - proliferative disorders originating in b , t , or natural killer ( nk ) lymphocytes . nhl has slowly grown from a rare cancer to the fifth most common cancer ( incidence of 19.1 per 100,000 in united states of america ( usa ) ) in the world over a period of 30 years . in india its incidence is on the upsurge with the current figure standing at 5.1 per 100,000 in urban registries . nhl is the 11 most common cancer in developed countries compared with developing countries , usually fatal , with a 5-year survival rate of less than 35% . nhl has been reported to occur as a primary lesion or can be associated with human immunodeficiency virus ( hiv ) infections since the beginning of the acquired immune deficiency syndrome ( aids ) epidemic in 1981 . the global burden of disease study 2010 estimates 1.5 million aids - related deaths in 2010 . aids - related lymphomas ( arls ) tend to present with high - grade b - cell histology , advanced - stage disease and an aggressive clinical course . with the introduction of highly active antiretroviral therapy ( haart ) in 1996 nhl is an important neoplasm , as it is an aids - defining condition . here , we present a rare case report of extranodal nhl in a known hiv patient . a male patient aged 68 years with a known hiv seropositivity since 5 years and who is on antiretroviral therapy reported to the department of oral and maxillofacial pathology , with the chief complaint of swelling in the right lower back region of the jaw since 2 months . patient also complained of loose molar teeth ( 46 , 47 and 48 ) in the same region . a detailed medical and dental history was taken . the patient complained of fever , night sweats , fatigue and loss of body weight . the patient was tested for cluster of differentiation ( cd ) 4/cd8 ratio , which was decreased ( 0.24 ) . the normal cd4 count is 500 - 1600 and cd8 count is 375 - 1100 . the normal cd4/cd8 ratio is below 15% . on extraoral clinical examination , the swelling was located on the right mandibular region leading to facial asymmetry [ figure 1 ] . intraorally there was an ulcero - proliferative growth in relation to 46 , 47 and 48 region on free and attached gingiva measuring about 6  4 cm in size , reddish in color , soft , sessile and oval in shape . a single right submandibular lymph node measuring about 1  1 cm , roughly oval shape was tender on palpation . orthopantamogram showed a diffuse radiolucent area in the lower right molar area in relation to 46 , 47 and 48 . patient showing growth on right lower back region with extraoral swelling on right cheek region ulcerative growth seen on the right buccal mucosa measuring about 5  6 cm in size extending anteroposteriorly from retromolar area to distal aspect of 46 region . an incisional biopsy was done along with the extraction of 46 , 47 and 48 teeth under local anesthesia ( 2% lignocaine with adrenalin , one in 80,000 dilution ) and the specimen was submitted for histopathological evaluation [ figures 3 and 4 ] . hematoxylin and eosin stained sections ( h and e ) showed surface - stratified squamous epithelium with ulceration . the connective tissue showed sheets of immature round tumor lymphoid cells of varying sizes and shapes , which were diffusely distributed in the connective tissue [ figure 5 ] . the round tumor cells showed dysplastic features like nuclear pleomorphism , prominent nucleoli and granular cytoplasm . two principle cell types observed were small cells with irregular or cleaved nuclear contours and scant cytoplasm referred to as centrocytes and larger cells with open chromatin , several nucleoli and modest amounts of cytoplasm referred to as centroblasts . there was evidence of increased and abnormal mitotic figures at focal areas . with these findings increased intraoral growth after 2 months incisional biopsy specimen along with extracted 46 , 47 and 48 teeth photomicrograph showing sheets of lymphoid cells ( h&e stain , 400 ) to identify the subtype of nhl , immunohistochemistry ( ihc ) was performed using cd45 , cd20 and cd3 markers . the positivity was indicated by intense nuclear brown staining on cell membrane surface [ figure 6 ] . the section was also positive for cd3 indicating t - cell lineage and negative for cd20 confirming that it was not of b - cell lineage [ figures 7 and 8 ] . the final diagnosis was confirmed as  extranodal peripheral t - cell non - hodgkin 's lymphoma . after 2 months , when the patient visited the hospital , the growth had increased and also had developed an extraoral swelling in relation to right lower jaw region . photomicrograph showing ihc positivity for cd45 with intense nuclear brown staining for cell membrane surface ( ihc stain , 400 ) photomicrograph showing negativity for cd20 staining ( ihc stain , 400 ) photomicrograph showing small cell t - lymphocytes positive for cd3 with intense nuclear brown staining for cell membrane surface ( ihc stain , 400 ) nhl though primarily a disorder of lymph nodes is also known to involve extranodal sites . when limited primarily to extranodal sites , it is referred to as primary extranodal lymphoma and accounts for 24 - 48% of all cases of nhl . most common site of extranodal disease in the western countries is reported to be gastrointestinal tract ( 4 - 20% of all nhl ) as compared to head and neck region , which is the commonest site ( 50.9% ) in india . different subsites include tonsil , orbit , nasopharynx , sinonasal tract , palate , eyelid , parotid gland and thyroid gland . the case presented here is also of male patient of age 68 years who was hiv positive and the growth was seen in lower right mandible in the retromolar area , which is a very rare site . a patient can come with oral lesion as initial sign and hence investigations should be performed to confirm diagnosis as nhl . precursor lymphoid neoplasms include both precursor b - lymphoblastic lymphoma / leukemia and t - lymphoblastic lymphoma / leukemia . both are essentially systemic disorders at presentation that may be manifested as lymphoma or leukemia . most patients present with lymphadenopathy but nodal versus extranodal should be examined , as the disease can occur in extranodal sites also . about a quarter of cases arising extranodally may be aggressive , but introduction of novel approaches and proper diagnosis will further improve prognosis . many classifications were given since 30 - 40 years , which can be difficult to interpret and reproduce . the improved classification systems real ( revised european - american lymphoma ) system in 2001 and modified world health organization ( who ) classification system in 2008 which are most accepted utilizes immunophenotyping by flow cytometry , cytogenetics and other molecular pathologic tools to designate particular lymphoma subtypes . the b- and t - cell neoplasms are stratified into precursor or lymphoblastic neoplasms and mature ( peripheral ) neoplasms . many forms of aids - nhl are characterized by the presence of recurrent genetic alterations , which may be due , in part , to errors in normal processes that occur in activated b cells , which involve modification of somatic dna , such as immunoglobulin gene ( ig ) , class - switch recombination ( csr ) and somatic hyper mutation ( shm ) . aids is able to produce dna double - strand breaks , resulting in widespread genome instability , which may contribute to some of the non - ig - related genetic modifications that are present in aids - nhl . the changes in hiv infection that result in chronic b - cell hyperactivation have the potential to contribute to the genesis of aids - nhl . the development of peripheral t - cell lymphoma is poorly understood and is thought to be associated with effector t cells involving chromosomal translocations . individuals without hiv infection generally have a greater number of cd4 cells than cd8 cells . as individuals get older , the immune system 's defense against pathogens is weaker and the cd4/cd8 ratio tends to decrease . individuals with autoimmune diseases tend to have an increased cd4/cd8 ratio , whereas those with viral infections have a decreased ratio . factors affecting cd4 count and subsequently the cd4/cd8 ratio are some viral infections , tuberculosis , corticosteroid use , seasonal / diurnal variations and variations in cd4 analyses . , in 2001 that the continued increased risk for nhl among haart - treated hiv - infected persons may be a reflection of incomplete immune reconstitution and possibly a chronic state of b - cell hyperactivation . antibody therapies for peripheral t - cell lymphoma are emerging as are approaches to improve antibody therapy such as conjugation with radioisotopes and chemotherapy agents . ihc plays a key role in the diagnosis and classification of hemato - lymphoid neoplasms . new cell and lineage markers are constantly being discovered and added to existing list of antibodies . the t - cell receptor associated protein f1 is expressed in approximately 75% of cases . in the present case , ihc using cd3 , cd20 and cd45 was done which confirmed the diagnosis of extranodal peripheral t - cell nhl . the cd20 marker is positive for reed sternberg cells and b - cell origin , which was negative in our case . the peripheral t - cell lymphoma associated with human t - cell lymphotrophic virus 1 and peripheral t - cell neoplasms are aggressive and the treatment is single agent and multi agent chemotherapy as well as several bio - immunotherapeutic agents ( such as alemtuzumab and denileukin diftitox ) a recombinant toxin containing interleukin 2 and truncated diphtheria toxin has also been used and are under investigation . the aggressive nhls are a group of malignancies with symptom being rapid onset and a short natural history . presenting features vary depending on the site affected . investigations are aimed at making an accurate diagnosis and the patient 's ability to tolerate aggressive therapy . the advent of monoclonal antibody therapy has improved outcomes in many b - cell malignancies . the aggressive lymphomas including many t - cell disorders carry a poor prognosis and new treatment strategies are needed .
non - hodgkin 's lymphoma ( nhl ) is primarily a disorder of lymph nodes , when limited primarily to extranodal sites it is referred to as primary extranodal lymphoma and accounts for 24 - 48% of all cases of nhl . 
 the development of peripheral t - cell lymphoma is poorly understood and is thought to be associated with effector t cells involving chromosomal translocations . 
 it has been suggested by few authors that the continued increased risk for nhl among highly active antiretroviral therapy ( haart)-treated human immunodeficiency virus ( hiv)-infected persons may be a reflection of incomplete immune reconstitution and possibly a chronic state of b - cell hyperactivation . here , we present one such rare case of extranodal nhl in a known hiv patient .