Subclinical TRAPS treated with canakinumab.

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare autoinflammatory disease characterized by recurrent episodes of fever and systemic inflammation. We describe the case of a 19-year-old patient who was referred to our attention with frequent subclinical TRAPS episodes characterized by mild arthralgias and crampy abdominal pain, without fever. Inflammatory markers, including serum amyloid A which increases the risk of long-term amyloidosis, were persistently high also when the patient was in good general conditions. Therapy with human anti-interleukin 1ß monoclonal antibody, canakinumab, led to disease control and normalization of the inflammatory markers, which are currently still normal. This clinical case supports the need to treat also subclinical TRAPS. In this respect, canakinumab is effective and reduces the risk of developing amyloidosis.

Focal nodular hyperplasia of the liver: an emerging complication of hematopoietic SCT in children.

Hepatic focal nodular hyperplasia (FNH) is a nonmalignant condition rarely affecting children previously treated for cancer, especially those who received hematopoietic SCT (HSCT). Some aspects of its pathogenesis still remain unclear and a strong association with specific risk factors has not yet been identified. We report here a single institution's case series of 17 patients who underwent HSCT and were diagnosed with FNH, analyzing retrospectively their clinical features and the radiological appearance of their hepatic lesions. We aimed to compare the diagnostic accuracy of ultrasound (US) and magnetic resonance imaging (MRI) and to explore the role of transient elastography (FibroScan) to evaluate the degree of hepatic fibrosis in FNH patients. Our analysis showed an association of FNH with age at transplant ⩽12 years (hazard ratio (HR) 9.10); chronic GVHD (HR 2.99); hormone-replacement therapy (HR 4.02) and abdominal radiotherapy (HR 4.37). MRI proved to be a more accurate diagnostic tool compared with US. Nine out of 12 patients who underwent FibroScan showed hepatic fibrosis. Our study points out that FNH is an emerging complication of HSCT, which requires a lifelong surveillance to follow its course in cancer patients.