P189. Lung-free lymphomatoid granulomatosis and Crohn's disease: An unknown asocciation
R. Estepa1, C. Medina2, A. Piñero3, E. Martin4, B. Melendez5, T. Castro3, A. Ferrer3, F. Garcia2, 1Hospital Jerez de la Frontera, Cadiz, Spain, 2Hospital Jerez de la Frontera, Surgery, Jerez de la Frontera, Spain, 3Hospital Jerez de la Frontera, Gastrointestinal, Jerez de la Frontera, Spain, 4Hospital Jerez de la Frontera, Hematology, Jerez de la Frontera, Spain, 5Hospital Jerez de la Frontera, Pathologia, Jerez de la Frontera, Spain
Lymphomatoid granulomatosis (LYG), is a rare condition. LYG is an Epstein–Barr virus (EBV)-related B-cell lymphoproliferative disorder characterized by a T-cell-rich polymorphous angiocentric and angiodestructive infiltrate. LYG affects the lungs predominantly. The digestive tract is rarely involved.
Patients with a primary or secondary immunodeficiency are at increased risk for LYG.
We report a case of LYG in a patient with Crohn's disease (CD). This description also identifies an EBV-driven B-cell lymphoma in a patient following methotrexate (MTX) immunosuppression for inflammatory bowel disease (IBD). We have proceeded to an extensive review of the literature and to the best of our knowledge no other cases of Lung-free LYG have been described in patient CD.
40 years old female. Diagnosed with Crohn disease when she was 20 years old, she was an estenotic colonic she needed right hemicolectomy and then she also needed transverse colectomy because of a new stenosis. Since then, she followed MTX treatment. She came for consult in July 2012 with malaise, weight loss, severe malnutrition, abdominal pain, inflammation in midline abdomen. A CT scan was performed: a subcutaneous collection was seen, and ileosigmoidy stenosis and bowel stenosis. Surgery was performed, ileosigmoid resection and protective ileostomy. There were no immediate complications after the procedure. She was admitted to the hospital again because of malaise, evening fever, pain in left hypochondrium, and dehydration. Pathology examination results were obtained, with the diagnosis of lymphomatoid granulomatosis.
The Hematology Department was consulted and Rituximab treatment was initiated (4 cycles). A new colon biopsy was obtained by colonoscopy and refractoriness was shown. Treatment with R-CHOPx6 was prescribed, obtaining parcial remission. Salvage therapy (R-ESHAPx2) was administrated, following autologous bone marrow transplantation.
Management of the disease was extremely challenging because of the overlapping of symptoms between Crohn's disease and Lymphomatoid Granulomatosis. Because it is a very rare condition, we needed to consult a renowned center in pathology. No lung affection has been shown during the development of the disease.
Immunosuppression produced by Crohn's disease treatment creates risks that must be assumed by patients and doctors that treat them. Complex clinical characteristics and with no response to conventional treatments must make us think about associated entities. Biologic treatments and more specific drugs could reduce the incidence of these serious diseases.