Oesophageal carcinoma and refractory idiopathic thrombocytopenic purpura: a challenging combination.

We report the case of a 72-year-old lady who presented with fatigue and bruising. Initial investigations were compatible with iron deficiency anaemia and idiopathic thrombocytopenic purpura (ITP). The anaemia and recent heartburn symptoms led to endoscopy, which revealed an extensive oesophageal squamous cell carcinoma. The tumour was inoperable due to local extension and the patient was not deemed fit enough for aggressive chemotherapy. A course of radical local radiotherapy was given. The patient's ITP, which had initially been responsive to high-dose intravenous immunoglobulin, subsequently became refractory to all therapeutic modalities. The patient developed significant dysphagia but therapeutic options were limited due to her severe thrombocytopenia. The patient eventually died from a combination of gastrointestinal and intrapulmonary haemorrhage. This case is of interest for two reasons. First, the development of refractory ITP appeared to mirror the progression of the carcinoma, and to our knowledge this is the first case of ITP associated with oesophageal carcinoma. Second, despite recent endoscopic advances in palliating oesophageal tumours, this case highlights the difficulties that can still occur.

The successful treatment of IgG4-positive colitis with adalimumab in a patient with IgG4-related sclerosing disease--a new subtype of aggressive colitis?

We present the case of a 16 year old girl who developed an aggressive colitis in the context of a prior biopsy proven autoimmune pancreatitis, which presented with obstructive jaundice at the age of 13 year. This history prompted prospective investigation and the discovery of compelling evidence to make a diagnosis of IgG4-related sclerosing disease with extra-pancreatic colonic involvement on the basis of raised serum IgG4 levels and a florid colonic IgG4 plasma cell infiltrate with over 20 IgG4 positive plasma cells/hpf. The colitis was resistant to conventional therapy but responded dramatically to treatment with the anti-TNFα monoclonal antibody, adalimumab. This is the first case to report both the effectiveness of adalimumab in treating IgG4 positive colitis in a patient with IgG4-related sclerosing disease, and to prospectively record resolution of an IgG4 positive colonic infiltrate with immunosuppression.