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Recurrent haemoptysis: a rare diagnosis of kaposiform lymphangiomatosis and review of literature
  1. Roukhama Fedidat1,
  2. Anna Elia2,3,
  3. Alon Hershko3,4 and
  4. Zvi Fridlender3,5
  1. 1Internal Medicine, Hadassah Medical Center, Jerusalem, Israel
  2. 2Pathology, Hadassah Medical Center, Jerusalem, Israel
  3. 3Faculty of Medicine, Hebrew University, Jerusalem, Israel
  4. 4Internal Medicine & Clinical Immunology, Hadassah Medical Center, Jerusalem, Israel
  5. 5Internal Medicine & Pulmonology, Hadassah Medical Center, Jerusalem, Israel
  1. Correspondence to Professor Zvi Fridlender; fridlender{at}


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.

  • malignant and benign haematology
  • respiratory medicine

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  • Contributors RF wrote this report and contributed to planning, collection of data, analysis and interpretation and review of the literature. AE contributed to pathological report, captions and figures. AH contributed to critical revision of the report. ZF contributed to critical revision and final correction of the report before publication.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.