celiac disease ( ced ) is a complex disorder of the immune system in which patients have a genetic predisposition and the ingestion of wheat gluten ( or related proteins ) triggers a deleterious immune response . tissue glutaminase binds these ingested peptides and presents them to t cells that lead to b cell production of autoantibodies . innate immunity is also involved targeting gliadin and tissue transglutaminase , causing inflammatory response with tissue damage . this immune response is complex and may lead to manifestations other than enterophaty : hepatitis , dermatitis , thyroid disorders and neuropathy . , enteropathy - associated t cell lymphoma ( eatl ) is a very rare malignancy ( incidence 0.5 to 1 per million ) . the 2008 world health organization classification of tumors of hematopoietic and lymphoid tissues recognizes two types of eatl , according to morphologic and genetic features . eatl type 1 is more frequently associated with ced , usually arising as late complication of refractory disease and carrying a dismal prognosis . several studies shown a trend for increasing incidence of ced and up to 20 or 30% of patients are diagnosed after the age of sixty we have recently observed an atypical clinical case , corresponding to a 69-year old caucasian female presenting with complicated celiac disease and eatl at diagnosis . the patient was referred to the department of hematology from the department of surgery following the histological examination of a partial enterectomy specimen , which unexpectedly revealed eatl in a background of ced . patient s previous medical history comprised obesity and several abdominal surgical procedures . in may 2008 , a laparotomy was performed to correct an incisional hernia and few months later she developed bowel obstruction requiring laparotomy . gradually the incisional hernia relapsed and in may 2013 a small bowel enterocutaneous fistula became apparent , entailing another laparotomy in which a partial enterectomy was performed . grossly , the specimen showed a diffuse whitish thickening of the intestinal wall and histological examination revealed typical features of eatl type 1 , as well as histological changes suggestive of ced in the adjacent mucosa ( figure 1 ) . the tumor immunophenotype was : cd2 + , cd3 + , and cd5 + ( low expression ) , cd8 + , cd30 + , cd4- , cd20- , cd15- , cd56- , alk- . in situ hybridization for ebv rna ( eber-1 ) was positive . at the time of admission in the department of hematology ( five months after the last surgery ) , the patient showed minor dehiscence of the abdominal incision with no further complaints . we sought for other clinical complaints that could suggest ced , but besides the occlusive episode , the patient referred regular bowel function and no systemic complaints . serologic testing for antigliadin antibodies and transglutaminase antibodies ( iga and igg ) were negative . the deamidated gliadin peptides assay was not performed because it is not available at our institution . staging computed tomography scan was consistent with localized disease and thus the patient was considered stage ii according to the lugano staging system for gastrointestinal lymphomas . although staging procedures are not standardized for this lymphoma , video capsule enteroscopy and 18f - fluorodeoxyglucose positron emission tomography are clinical options . the patient was treated with six cycles of chemotherapy according to the chop ( cyclophosphamide , doxorubicin , vincristine and prednisolone ) protocol and referred for nutritional counseling . between chemotherapy cycles the patient received hematopoietic growth factor ( g - csf ) prophylactic treatment . the minor dehiscence of the abdominal incision showed on several occasions signs of mild infection . she had regular nursing wound care and often needed oral antibiotic prescription , according to the microbiology swabs . the patient never experienced neutropenic fever or other signs of severe infection . at the end of chemotherapy the patient was in complete remission : both the computed tomography scan and biopsy of enterocolic anastomosis were negative for neoplasia . high - dose chemotherapy and autologous stem cell transplantation was not pursued since this was an elderly patient , prone to complications during this aggressive treatment and had localized disease at diagnosis . it has been difficult to alter the nutritional habits of this elderly patient , but since the eatl diagnosis the patient is receiving nutritional counseling to follow a gluten - free diet . in the context of ced , this patient displayed a very atypical clinical picture , presenting with enterophaty - related lymphoma concomitant with histological changes suggestive of ced in the adjacent mucosa , without prior symptoms suggestive of ced . one may question if the previous abdominal relapsing complications that required multiple laparotomies across several years were due to silent ced . this atypical clinical presentation contributed to diagnosis delay and lack of specific measures that could have contributed to prevent the evolution towards eatl . the patient was exposed to gluten during all her lifetime and it is widely acknowledged that a glutenfree diet effectively prevents the development of eatl in patients with overt ced . in this clinical case there was no formal indication to institute a gluten - free diet previous to the diagnosis of lymphoma , since the diagnosis of ced was simultaneous to that of eatl . one may question whether silent ced patients would benefit from an earlier institution of gluten - free diet in order to prevent those events.enteropathy-associated t cell lymphoma is frequently diagnosed after pathologic evaluation of a resected specimen of small bowel due to intestinal obstruction or perforation . complications such as perforation , infection , fistulae and poor nutritional status related with chronic malabsorption may postpone systemic treatment . this plays a relevant role in defining part of the poor prognosis of this disease . nutritional support , sometimes with parenteral nutrition , and rigorous control of infectious complications are critical issues in the management of these patients . although she had a minor dehiscence of the abdominal incision she was able to tolerate chemotherapy . usually the treatment consists of a combination chemotherapy regimen used for other aggressive t cell lymphomas . this patient was not a candidate for autologous hematopoietic transplantation , but some studies ( based in small number of patients ) suggest survival benefit with transplantation in first remission when patients are eligible . generally eatl is an aggressive lymphoma with high - grade histology and poor prognosis , usually worse than other intestinal lymphomas . the major determinants of prognosis are stage at presentation and clinical status to allow for systemic treatment . occasional patients with localized disease may be cured but many patients will die from complications of multifocal intestinal perforation or systemic progression . the five - year overall survival rate with anthracycline - based chemotherapy is approximately 10 to 20 percent . this case draws our attention to clinically silent ced , which represents a diagnostic challenge . thus , this should be kept in mind whenever a patient presents with abdominal relapsing complications which are otherwise unexplained .
celiac disease is an autoimmune disorder in which a genetic predisposition and the ingestion of wheat gluten triggers a deleterious immune response . 
 this response is complex and may lead to manifestations other than enteropathyha : hepatitis , dermatitis and neuropathy . 
 there is higher risk for neoplasia . 
 we observed an atypical case , corresponding to a 69-year old female presenting with complicated celiac disease . 
 the patient was referred following the histological examination of an enterectomy specimen , which unexpectedly revealed an enteropathy - associated t cell lymphoma in a background of celiac disease . 
 patient s previous medical history comprised several abdominal surgical procedures , without other prior symptoms suggestive of celiac disease . 
 indeed , the patient was obese and no signs of malabsortion were apparent . 
 this case draws our attention to clinically silent celiac disease , which represents a diagnostic challenge . 
 thus , this should be kept in mind whenever a patient presents with abdominal relapsing complications , otherwise unexplained .