Improvement of disseminated lymphangiomatosis with recombinant interferon therapy

Lower-Extremity Lymphangiomatosis

“…Interferon-alpha is believed to have some antiproliferative and anti-angiogenic properties, which seem to make it a reasonable choice for the treatment of this disorder 17 . Some of the reports described extremely ill patients with pulmonary involvement who had correspondingly mixed results; but, clearly, as was seen in our patient, the use of this agent can be quite effective in patients with bone and soft-tissue disease 11,17,26 . Some of the reports described extremely ill patients with pulmonary involvement who had correspondingly mixed results; but, clearly, as was seen in our patient, the use of this agent can be quite effective in patients with bone and soft-tissue disease 11,17,26 .…”

Thoracic lymphangiomatosis with massive chylothorax after a tumor biopsy and with disseminated intravenous coagulation — lymphoscintigraphy, an alternative minimally invasive imaging technique: Report of a case

“…Recently, interferon-α therapy has been reported to yield a successful outcome without any life-threatening side effects. 3,24 In the present case, severe respiratory distress restricted the use of adequate radiation therapy or interferon therapy. It might, therefore, have been advantageous to employ these therapies immediately upon onset.…”

A gene missense mutation in diffuse pulmonary lymphangiomatosis with thrombocytopenia

“…Two cases of DPL involving the spleen with thrombocytopenia and DIC showed improvement after partial splenic embolization and splenectomy; therefore, such cases may be related to lymphoma involving the spleen. [ 11 , 12 ] For the patient in this report, the lesions involved the lungs, the pericardium, and the pleura, but the spleen was not involved; in addition, platelet counts were reduced. A mutation in the TNFRSF13B gene (c.431C > T, p.Ser144Leu) was found in the current patient by exome sequencing, and a gain-of-function mutation in TNFRSF13B was reported as a candidate for the predisposition to familial or sporadic immune thrombocytopenia.…”