Kaposiform lymphangiomatosis (KLA) is a rare clinicopathological entity among lymphatic anomalies. The main involved sites are the mediastinum and the lungs but the disease can also affect multiple extrathoracic organs. Little is known about the pathophysiology, the natural history, the treatment response and the long-term outcome of this disorder. KLA is typically diagnosed in childhood. We present here the case of an adult man with 13 years recurrent episodes of haemoptysis who was finally found to suffer from KLA. Following this, we present a comprehensive review of the literature.
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- malignant and benign haematology
- respiratory medicine