Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease

Michio Ozeki, Akihiro Fujino, Kentaro Matsuoka, Shunsuke Nosaka, Tatsuo Kuroda, Toshiyuki Fukao

研究成果: Article

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Background: Complex lymphatic anomalies are intractable lymphatic disorders, including generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD), and kaposiform lymphangiomatosis (KLA). The etiology of these diseases remains unknown and diagnosis is confused by their similar clinical findings. This study aimed to clarify the differences in clinical features and prognosis among GLA, KLA, and GSD, in Japanese patients. Procedure: Clinical features, radiological and pathological findings, treatment, and prognosis of patients were obtained from a questionnaire sent to 39 Japanese hospitals. We divided the patients into three groups according to radiological findings of bone lesions and pathology. Differences in clinical findings and prognosis were analyzed. Results: Eighty-five patients were registered: 35 GLA, 9 KLA, and 41 GSD. Disease onset was more common in the first two decades of life (69 cases). In GSD, osteolytic lesions were progressive and consecutive. In GLA and KLA, 18 patients had osteolytic lesions that were multifocal and nonprogressive osteolysis. Thoracic symptoms, splenic involvement, and ascites were more frequent in GLA and KLA than in GSD. Hemorrhagic pericardial and pleural effusions were more frequent in KLA than GLA. GSD had a significantly favorable outcome compared with combined GLA and KLA (P = 0.0005). KLA had a significantly poorer outcome than GLA (P = 0.0268). Conclusions: This survey revealed the clinical features and prognosis of patients with GLA, KLA, and GSD. Early diagnosis and treatment of KLA are crucial because KLA has high mortality. Further prospective studies to risk-stratify complex lymphatic anomalies and optimize management for KLA are urgently needed.

元の言語English
ページ(範囲)832-838
ページ数7
ジャーナルPediatric Blood and Cancer
63
発行部数5
DOI
出版物ステータスPublished - 2016 5 1

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology

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