Interferon alpha 2b treatment in an eleven‐year‐old boy with disseminated lymphangiomatosis

Abstract

Disseminated lymphangiomatosis is a rare disease mostly observed in children and young adults. If no surgical removal can be achieved, the prognosis is poor, especially for patients with thoracic localization and pleural effusions. Next to pleural drainage, therapeutic options include radiotherapy, local, and systemic pharmacotherapy. An 11-year-old boy presented with disseminated lymphangiomatosis involving thorax with massive pleural effusions, retroperitoneum, and bones. In immunohistochemical analysis, the tissue biopsy stained positive for vascular endothelial growth factor-receptor 3 (VEGFR-3). The patient has been treated with interferon alpha 2b for 2 years, and achieved a good clinical and radiological response.

Sirolimus for the treatment of children with various complicated vascular anomalies

“…Treatment of life-threatening hemangiomas and other vascular tumors with cytostatics, such as cyclophosphamide and vincristine, not only yields a variable response but also significant short-term and long-term side effects, especially in children under 18 months of age [6,16,18]. Interferon-α is known to suppress angiogenic growth factors' activity and has been applied successfully in different vascular anomalies, which failed to respond to other therapies [5,24,29]. A serious limitation is the danger of irreversible spastic diplegia in infants [27].…”

Unique Diagnostic Features and Successful Management of a Patient With Disseminated Lymphangiomatosis and Chylothorax

“…18,19 The agent most often reported to have efficacy in controlling the growth of lymphatic tumors has been interferon-a. [20][21][22][23][24] The optimal duration of treatment is unclear, with courses as long as 40 months having been described. 20 Interferon is generally well tolerated, with the most common toxicities being mild flulike symptoms, neutropenia or anemia, and transient liver enzyme elevation.…”

Serious complications of pulmonary biopsy in a boy with chylopericardium and suspected pulmonary lymphangiomatosis

“…Surgical interventions, such as excision of a localised lesion, pleurodesis, pleurosclerosis, pleuroperitoneal shunting, chest wall resection or thoracic duct ligation in the case of thoracal involvement have been applied with differing results 14 , 21 , 23 – 27. Systemic treatment with corticosteroids, immunomodulating agents and chemotherapy have shown varying results 2 , 9 , 12 , 24 , 26 , 27. Interferon α, a cytokine with antiangiogenic activity, appears to be beneficial and has been used for the treatment of lymphangiomatosis for almost 20 years.…”

“…Interferon α, a cytokine with antiangiogenic activity, appears to be beneficial and has been used for the treatment of lymphangiomatosis for almost 20 years. Clinical improvement has been reported in the majority of cases, although the optimal treatment duration remains unclear and ranges from 3 weeks to several years 7 , 21 , 24 , 26 , 27…”