Erdheim-Chester disease: A case report

Introduction. Erdheim-Chester disease (ECD) is a rare non-Langerhans-cell histiocytosis. About 500 cases are published so far. It is multisystemic disease characterised by bilateral symmetric long bones sclerosis. Main histopathological finding is accumulation of big foamy histiocytes, immunohistochemically positive to CD68, and negative to S-100 and CD1a. There are no guidelines that reliably identify population that requires therapy, but symptomatic ECD, organ failure and central nervous system involvement re-quire treatment. Case report. We described a patient with a multisystemic form of ECD affecting long bones, the hypophysis, abdomen, and the peripheral nerves. Five years after initial symptoms ECD was suspected. Prednison was initiated, 60 mg once a day. After obtaining the diagnosis of ECD, interferon alpha 2A was introduced, but soon after stopped due to severe side effects. Considering that histiocytes were positive to platelet derived growth factor receptor alpha (PDGFR alpha) imatinib mesylate was started, but after two months stopped due to no clinical and radiological improvement. The disease was worsening and the patient died. Conclusion. We described the patient with intraperitoneal form of ECD, without cardiac and pulmonary involvement. There are several important issues: the diagnosis of ECD could be difficult to make, three treatment regimens were included and the patient died nine years after the initial symptoms due to indolent course of the disease and unsuccessful treatment

Autoimmune pancreatitis type 1 and type 2: A report of two cases

Introduction. Autoimmune pancreatitis is a disease associated with autoimmune mechanisms, clinically manifested mostly as obstructive icterus with or with no entire or partial enlargement of the pancreas, histological lymphoplas-mocytic infiltration, fibrosis or granulocytic epithelial lesions with a favourable therapeutic response to the application of corticosteroids. Type 1 autoimmune pancreatitis is a systemic disease befalling the group of IgG4-related diseases in contrast to type 2 which is specific for pancreas disease. Case report. We presented two cases. The first one was a 64-year-old male patient with autoimmune pancreatitis complaining of abdominal pain, weight loss, weakness and exhaustion. Clinical examination showed a rare IgG4 autoimmune pancreatitis. The second one was a 37-year-old male patient complaining of abdominal pain with diarrhea. The diagnosis made revealed the presence of type 2 autoimmune pancreatitis. Following the diagnosis, immunosuppressive therapy was administered to both patients leading to the improvement of their general condition. Conclusion. Autoimmune pancreatitis is a rare disease, sometimes not easy to differ from pancreatic tumor or bile duct tumor with poor prognosis. Thus, early recognition of the disease is very important, since adequate treatment significantly increases the course and the outcomes of the disease