Treatment-Refractory, Primary Immune Thrombocytopenic Purpura in a Patient with Celiac Disease

Patient: Male, 27-year-old Final Diagnosis: Immune thrombocytopenic purpura (ITP) Symptoms: Bleeding • purpura Medication: Azathioprine • eltrombopag • fostamatinib • intravenous immunoglobulin • IVIG • prednisone • rituximab • steroids Clinical Procedure: EGD • PET-CT • plasmapheresis • splenectomy Specialty: Gastroenterology and Hepatology • Hematology • General and Internal Medicine OBJECTIVE: Unusual clinical course BACKGROUND: Immune thrombocytopenic purpura (ITP) is primarily caused by antibody-mediated destruction of platelets. Alterations in immune homeostasis can induce loss of peripheral tolerance and promote the development of self-reactive antibodies. Primary ITP is the diagnosis of exclusion made after the extensive work-up rules out other possible causes of thrombocytopenia. The association between the ITP and other autoimmune disorders is well-established. In recent years, increasing attention has been directed toward the association between celiac disease (CD) and ITP. CASE REPORT: A 27-year-old man with a history of primary ITP presented with an occasional nosebleed, 1 episode of rectal bleeding, and easy bruising. The patient was later found to have high titers of TTG-IGA and endomysial IGA levels consistent with CD. Our patient not only failed to improve with the gluten-free diet, but also failed multiple lines of treatment including steroids, IVIG, rituximab, eltrombopag, and even a non-traditional treatment for ITP (azathioprine and plasma exchange). The patient’s CD-related antibody titers remained elevated. CONCLUSIONS: It is possible that in certain cases the alteration of immune response caused by CD with a concurrent elevation of CD-related antibodies can make ITP refractory to all medical management. Whether or not this refractoriness to treatment is related to the persistently elevated antibody titers of CD or unknown genetic relationship between ITP and CD remains not entirely clear and warrants further molecular, immunologic, and genetic analysis.