Abstract
Celiac disease has been associated with a number of immune-mediated hematologic disorders, including idiopathic or immune thrombocytopenic purpura (ITP). Review of the literature suggests that children are particularly affected, often with overt skin findings, but in this report, a 39-yr old male with biopsy-defined celiac disease was detected with a persistent immune-mediated reduction in his platelet count despite treatment and follow-up for more than a decade. Although the celiac disease resolved with a strict gluten-free diet, there was no effect on the ITP. Experience suggests that ITP may be rarely appreciated in adults (compared to children) because it may be limited in severity and occurs without clinically obvious purpura or evidence of bleeding, even with diagnostic or therapeutic biopsy procedures. A subsequent retrospective review of over 300 celiac disease patient records from a single teaching hospital clinical practice experience spanning almost 40 years confirmed no additional cases of thrombocytopenia. ITP appears to be rarely associated with celiac disease in adults and, in spite of resolution of the enteropathy, did not appear to respond to a gluten-free diet, even after more than a decade of dietary treatment.
Highlights
Celiac disease is an immune mediated small intestinal mucosal disorder, often associated with diarrhea and weight loss [1]
Celiac disease and long-standing persistent thrombocytopenia were documented in this patient over more than a decade
The gluten-free diet appeared to have no effect on the platelet count despite more than a decade of gluten-free diet treatment
Summary
Celiac disease is an immune mediated small intestinal mucosal disorder, often associated with diarrhea and weight loss [1]. The disorder responds to treatment with a strict gluten-free diet. Different immune-mediated disorders may be associated with celiac disease. In one report [5], long-term therapy with a gluten-free diet was reported to be associated with resolution of thrombocytopenia further suggesting a significant immune-mediated linkage. Celiac disease may result in thrombocytopenia through other mechanisms, not necessarily directly related to an immune-mediated pathogenesis. Other concomitant or coincidental causes may be present, including immunoglobulin A deficiency and either a drug-induced or a viral infection associated with thrombocytopenia [1,6]. The present report documents persistent immune thrombocytopenia in an adult with celiac disease. The enteropathy responded completely to a gluten-free diet alone, the thrombocytopenia persisted. A subsequent retrospective evaluation of over 300 additional adults with celiac disease failed to detect thrombocytopenia. In the present patient here, diagnostic and therapeutic biopsy studies were not prohibited
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