Fig. 1

Radiologic findings and pathological features in KLA (Patient #3). a Unenhanced CT scan of the chest demonstrates thickening of the interlobular septa. Axial T2-weighted thoracic MRI shows pericardial effusion and high-signal abnormality in the mediastinum that extends along the bronchovascular bundles (b). Axial T2-weighted MRI of the abdomen shows multiple high-signal splenic lesions (c). Macroscopic view of the spleen excised from the patient (d). Multiple, small, reddish blue nodules are noted on the surface of the spleen. Hematoxylin-eosin staining shows dilated lymphatic channels and dispersed small irregular cellular clusters within the splenic parenchyma. The clusters are comprised of spindle-like cells without distinct lumens e. The spindle-like cells are immunopositive for CD31 (F) and D2–40 g. 6 months after splenectomy, MRI revealed a significant involution of the lesion h