A 36-year-old man with a 3-year history of celiac disease comes to the physician with fatigue, low-grade fevers, abdominal pain, and diarrhea for 3 months. He was symptom free for 2 years when he followed a strict gluten-free diet. However, in the past year, he developed symptoms twice due to poor dietary compliance. The patient's other medical problems include type I diabetes mellitus and depression. His father died of colon cancer at age 55. His mother has diabetes, and a sister has celiac disease.
His temperature is 38.3° C (101° F), blood pressure is 110/80 mm Hg, and pulse is 80/min. The abdomen is soft but tender in the periumbilical area. There is no rebound tenderness or rigidity. Rectal examination shows dark stools positive for blood.
Laboratory results are as follows:

Which of the following is the most likely cause of this patient's current symptoms?

Question

A 36-year-old man with a 3-year history of celiac disease comes to the physician with fatigue, low-grade fevers, abdominal pain, and diarrhea for 3 months.  He was symptom free for 2 years when he followed a strict gluten-free diet.  However, in the past year, he developed symptoms twice due to poor dietary compliance.  The patient's other medical problems include type I diabetes mellitus and depression.  His father died of colon cancer at age 55.  His mother has diabetes, and a sister has celiac disease.
His temperature is 38.3° C (101° F), blood pressure is 110/80 mm Hg, and pulse is 80/min.  The abdomen is soft but tender in the periumbilical area.  There is no rebound tenderness or rigidity.  Rectal examination shows dark stools positive for blood.
Laboratory results are as follows:

Which of the following is the most likely cause of this patient's current symptoms?

Quizplus · Accepted Answer

11ec193a_980d_edff_853d_3360511fb1a1_MD0002_00 This patient's presentation (3 months of fevers, abdominal pain, melena, and diarrhea) is concerning for intestinal lymphoma, specifically enteropathy-associated T cell lymphoma (EATL). This is a rare and aggressive lymphoma that is seen more commonly in celiac disease patients with poor dietary compliance. Patients present with abdominal pain, hepatosplenomegaly, ascites, duodenal masses, B symptoms (eg, fever, night sweats, and weight loss), and gastrointestinal bleeding. Some patients may present with obstruction or perforation. The tumor is commonly located in the proximal jejunum and less frequently in the stomach, small bowel, or colon. A strict gluten-free diet also appears to reduce the incidence of EATL. EATL should also be suspected in patients with refractory sprue despite good dietary compliance and failure of treatment with glucocorticoids. It may also be helpful to test for underlying celiac disease in all patients with a T cell lymphoma with or without gut involvement as celiac disease is undiagnosed in many individuals. However, EATL is uncommon in individuals diagnosed with celiac disease at a young age (< 33 years). Diagnosis is confirmed on pathology of resected tumor, which demonstrates multiple jejunal ulcers often with gut perforation. EATL has a poor prognosis and treatment consists of combination chemotherapy used for other aggressive T cell lymphomas. (Choice A) Clostridium perfringens usually presents with self-limited watery diarrhea and enteritis necroticans (hemorrhagic necrosis of the jejunum), both of which would not cause symptoms for 3 months. (Choice B) Poor patient compliance with a gluten-free diet is the most common cause of recurrence of bowel symptoms in celiac disease. However, poor compliance and disease recurrence usually do not cause persistent fever, as seen in this patient. (Choice D) Patients with celiac disease may have coexisting pancreatic insufficiency. However, bloody stools and fever are not typically seen in chronic pancreatic insufficiency. (Choice E) Refractory sprue is defined as persistent intestinal pathological changes despite a strict gluten-free diet for 12 months. However, this would not explain this patient's B symptoms or bloody diarrhea. Educational objective: Enteropathy-associated T cell lymphoma is an aggressive lymphoma commonly seen in patients with celiac disease and poor dietary control. Patients present with abdominal pain, B symptoms, hepatosplenomegaly, ascites, bowel obstruction/perforation, and/or gastrointestinal bleeding. Diagnosis is confirmed by pathology of resected tumor. Prognosis is poor, and treatment involves combination chemotherapy. __________ References: Enteropathy-associated T-cell lymphoma: epidemiology, clinical features, and current treatment strategies. (http://www.ncbi.nlm.nih.gov/pubmed/21912848) Enteropathy-associated T-cell lymphoma. (http://www.ncbi.nlm.nih.gov/pubmed/20655757)

A 36-Year-Old Man with a 3-Year History of Celiac Disease