1/5. Lymphocytic hypophysitis associated with dacryoadenitis: an autoimmunologically mediated syndrome.We report a rare case of lymphocytic hypophysitis followed by dacryoadenitis. Lymphocytic hypophysitis is a rare disease that can easily be mistaken for neoplastic proliferation. Because combination with rheumatoid arthritis, thyroiditis, or pernicious anemia is frequent, an immunological pathogenesis is likely.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. Idiopathic enterocolic lymphocytic phlebitis: a rare cause of ischemic colitis.We report on a 74-year-old female patient who was admitted to the hospital because of abdominal pain. She underwent a colonoscopy and a stenosing mass was found in the cecum. Histologic findings in the biopsy specimens were consistent with ischemic colitis. Due to clinical symptoms and the endoscopic and radiologic findings that roused the suspicion that the patient was suffering from a malignant tumor, a right hemicolectomy was performed. histology of the resection specimen disclosed an inflammation of the veins. It was characterized by a predominantly lymphocytic infiltration of the vessels affecting the veins of the colonic wall and the mesentery. Furthermore, secondary thrombosis with focal venous occlusion was observed. The colon showed extensive ischemic colitis with focal transmural coagulation necrosis. The disease was considered to be idiopathic lymphocytic phlebitis, which is a rare disease of unknown origin. Our patient is well and alive after more than 1 year, supporting the notion that the disease shows a benign course after surgery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/5. Persistent polyclonal B lymphocytosis: morphological, immunological, cytogenetic and molecular analysis of an Italian case.We describe a case of persistent polyclonal B-cell lymphocytosis (PPBL) studied by morphological, immunological, cytogenetic and molecular analysis. PPBL is a rare lymphoproliferative disorder with an unclear natural history. Although a few cases of malignancies are observed during PPBL, this disorder is usually considered to be an indolent syndrome. A longer follow-up in a larger number of patients is needed in order to clarify the natural history of PPBL and its potential to transform into a malignancy. As PPBL is a rare disease, establishing an international PPBL registry could be the most effective way to understand the natural history of this disease and to discover its etiologic factors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. Collagenous gastritis associated with lymphocytic gastritis and celiac disease.Collagenous gastritis is a rare disorder, with only 8 cases reported in the literature, 2 in children and 6 in adults. We report an additional case of collagenous gastritis in a 42-year-old man with celiac disease. A thickened (>10 microm) subepithelial collagen band with entrapped capillaries, fibroblasts, and inflammatory cells was seen in the stomach, associated with lymphocytic gastritis. The duodenal mucosa showed severe villous atrophy but no subepithelial collagen deposition. No evidence of lymphocytic or collagenous colitis was found in the colon. The patient became symptom-free on a gluten exclusion diet and showed partial improvement of histopathologic findings after 3 months. Collagenous gastritis is a rare disease, but a wider recognition of its histopathologic features and clinical associations may bring more cases to light and provide additional clues in determining its etiology and pathogenesis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. Idiopathic pure red cell aplasia: first report on CD8 positive lymphocytosis in bone marrow biopsy sections.There is no information in the literature regarding the lymphocyte content or type in bone marrow biopsies from patients with "idiopathic" pure red cell aplasia (PRCA). This report describes the bone marrow biopsy sections of a patient with PRCA. A diffuse CD3 positive (CD8 positive, granzyme B negative) lymphocytosis of approximately 1500/mm3 was revealed by immunohistochemical staining. The extent of the T cell increase was not evident from morphological examination of the bone marrow aspirate or biopsy, from flow cytometric analysis of the aspirate, or from the peripheral blood lymphocyte count. Therefore, immunohistochemical analysis should be performed routinely in this rare disease and the data acquired may help to inform the choice of treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |