Association of protein-losing enteropathy caused by eosinophilic gastroenteritis with essential thrombocytosis: case report.

Protein-losing enteropathy is not a single disease; it is an atypical manifestation of other disease processes and is marked by excessive loss of protein into the gastrointestinal tract. These diseases can be classified into 3 large groups according to the protein-loss mechanism: ulcerated lesions with protein exudation (eg, peptic ulcer, inflammatory bowel disease, and gastric carcinoma), mucosal lesions without ulceration, but with increased permeability (eg, Ménétrier’s disease, celiac sprue, and eosinophilic gastroenteritis), and obstructive diseases (eg, lymphoma), as well as primary disorder of the lymphatic system (eg, intestinal lymphangiectasia). One of the several causes of protein-losing enteropathy is eosinophilic gastroenteritis. This is a rare disease characterized by infiltration of eosinophils from the mucosa to the serosa layer, mainly in the stomach and the small bowel. Eosinophilia and anemia are common hematological manifestations, but platelet abnormalities have not been reported. Essential thrombocytosis is a rare myeloproliferative disorder. High platelet count, which can be as high as more than 1 million platelets/mm in peripheral blood, is the hallmark of this disorder. The disease can progress with thrombotic and hemorrhagic episodes. Essential thrombocytosis is not a cause of protein-losing enteropathy, and its association with eosinophilic gastroenteritis has not been reported. We report a rare case of a concurrent manifestation of eosinophilic gastroenteritis and essential thrombocythemia. A 56-year-old brown-skinned man from São Paulo was admitted to Hospital das Clínicas, the São Paulo University Medical School Hospital in January 2004, with anasarca and ischemia of the fourth and fifth toes of his left foot. The patient reported progressive increase of abdominal girth and lower extremities as well as facial edema for 5 months, and pain in and darker skin of the fourth and fifth toes of his left foot over the previous 10 days. He also reported hyporexia and a 18-kg body weight loss associated with dyspepsia, flatulence, and episodes of hiccups. He had no complaints of urinary alterations, dyspnea, abdominal pain, intermittent claudication, altered bowel habits, or altered stool. The patient also reported that he had been hypertensive for 10 years and had made irregular use of propranolol. A former smoker (from 14 to 32 years of age), he had allergic rhinitis. He had no history of diabetes, excessive alcohol consumption, or blood transfusions. On physical examination, his overall condition was good, with normal colored mucosa; eupnea; body weight, 70 kg; heart rate, 80 beats/minute; and blood pressure, 150/ 100 mm Hg. There was evidence of pleural effusion at the base of the left lung, moderate ascites, moderate edema of the lower extremities, and mild facial edema. The fourth and fifth toes of his left foot were sore, with darker coloration (Fig. 1). His peripheral pulses were symmetric with normal amplitudes.