Langerhans Cell histiocytosis is a rare proliferative histiocytic disorder in which pathologic Langerhans cells accumulate in a variety of organs. The clinical presentation, evolution and therapeutic options are highly variable. Because of its relative rarity and the broad clinical spectrum, the diagnosis of Langerhans cell histiocytosis is often delayed or missed. At present, many questions with respect to aetiology, pathogenesis and treatment remain unanswered. In the present article we want to raise the awareness of this rare disease in adults and its diversity by the means of two case reports. In addition, the clinical manifestations, diagnosis and the current management are reviewed.
Three siblings with idiopathic hemochromatosis are presented. They are remarkable because of the particularly benign disease and absence of typical HLA phenotype in the index case. In only one of the patients is the glucose tolerance disturbed. This case also has an HLA A3 phenotype. A typical hemochromatosis arthropathy is described in two patients. Following venesection a subjective improvement of the arthritis was reported by one patient.
In an effort to map the use of interleukin-2 (IL-2) treatment in patients with clear cell renal cell cancer (RCC) in Belgian hospitals, 44 cases were registered from 9 hospitals between February 2003 and June 2006. It was demonstrated that the majority of these patients were treated with subcutaneous (SC) IL-2. Other methods such as the inhalation of the drug in case of intrathoracic disease or high dose intravenous (IV) administration were much less frequent (3 and 0 cases in this registry, respectively). The results of antitumour activity (around 16% partial response-absence of complete responses) and toxicity of this drug correlate with observations from the literature with the SC administration. In view of the poor results and tolerance with the currently used cytokines (IL-2 or interferon-alfa), much hope is directed towards the development of the novel targeted drugs like sunitinib or sorafenib used alone or in combination with cytokines in this disease.
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