“…The differential diagnosis of diffuse interstitial pulmonary disease with splenomegaly in childhood encompasses other vascular diseases (lymphangiectasis, lymphangioleiomyomatosis, hemangioendothelioma, angiosarcoma, and pulmonary venoocclusive disease), infections, connective tissue diseases, cystic ®brosis, benign disorders such as histiocytosis, and malignancies such as leukemia or lymphomas [4,11,22]. If an interstitial pulmonary disease is accompanied by thrombocytopenia, anemia, and consumptive coagulopathy or other organ involvement, angiomatosis should be included in the differential diagnosis.…”