This T-cell lymphoma may manifest itself as “refractory sprue.” In its evolution, enteropathy-type T-cell lymphoma first features small-bowel lymphoepithelial lesions in a background of celiac-type histology. The latter neoplasm is virtually confined to patients with CD, many of whom have had a short adult-onset course of CD. Increased risk for the development of non-Hodgkin lymphoma, in particular entero-pathy-type T-cell lymphoma. Increased risk of primary small-bowel adenocarcinomas ( 1, 2, 3, 13, 14, 15) Increased risk of esophageal squamous cell carcinoma (perhaps related to a deficiency of trace metals such as zinc) ( 1, 2, 3, 13, 14, 15) In comparison to control populations, the following abnormalities have been defined: If untreated, CD (whether florid or subtle) is associated with serious long-term complications. In addition, CD has recently been suggested to be the actual cause of symptoms in some patients diagnosed with irritable bowel syndrome ( 11, 12). The major clinical findings in atypical, “late-onset,” or latent CD include short stature, infertility, peripheral neuropathies, iron and/or folate deficiency, osteoporosis, recurrent abdominal pain (children), “indigestion” (adults), and dental enamel defects diarrhea is usually minimal or absent, and malabsorption is detected only by specific testing ( 1, 2, 3, 4, 5, 6, 7, 8, 9, 10). In fact, in some series, about 25% of patients ultimately shown to have CD are diagnosed after the age of 45 to 50 years. However, as a result of recent serological and biopsy investigations, it has become clear that CD may be a more subtle disease, with protean manifestations, that is first diagnosed in early adulthood or later. Because the onset of classic cases is typically in childhood, failure to thrive is a major presenting complaint. The classic presentation of CD is a severe malabsorption syndrome with diarrhea, steatorrhea, and weight loss.
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