Kaposiform Lymphangiomatosis, a Newly Characterized Vascular Anomaly Presenting with Hemoptysis in an Adult Woman.
[lymphangioleiomyomatosis]
Disorders of the pulmonary lymphatic system include macro and microcystic lymphatic malformations, primary or secondary lymphangiectasias, generalized lymphatic anomalies (GLA), diffuse pulmonary lymphangiomatosis (DPL), and combinations of lymphatic and other tissue anomalies including lymphangioleiomyomatosis (LAM). We report a case of a patient with a newly defined entity classified as kaposiform lymphangiomatosis (KLA). A 50 year old nonsmoking Hispanic female presented with a 20 year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. Laboratory evaluation demonstrated a low normal platelet count, elevated D-Dimer, low normal fibrinogen and elevated fibrin split products. Chest computerized tomography scan showed enlarged hypodense lymph nodes in the mediastinum and hila, and peribronchovascular thickening, without evidence of cystic parenchymal lesions. MRI of the chest showed a cystic, septated appearance of the mediastinal lymph nodes with heterogeneously increased T2 and decreased T1 signal intensity. Fiberoptic bronchoscopy revealed hyperemic mucosa with granular appearance secondary to submucosal infiltrative process. Pathological specimens revealed dilated, malformed lymphatic channels within the pleura, pulmonary septa, and bronchovascular bundles, and foci of perilymphatic and intralymphatic spindle cells which reacted with the PROX-1 immunostain. The morphology and immunohistochemistry results were consistent with a diagnosis of KLA. KLA is a recently described entity amongst lymphatic anomalies. Clinically, patients have a generalized dysplastic lymphatic process with involvement of the mediastinum, lungs, retroperitoneum, spleen, bones, soft tissue, and skin. Mortality is associated with progressive pulmonary disease and serosal hemorrhage. Medical treatments reported include Sirolimus, and anti-angiogenic agents. Our patient received Sirolimus and will be followed in our clinic.
