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 T 2 and decreased T 1 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 .

Diseases
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Diseases presenting "enlarged hypodense lymph nodes in the mediastinum" symptom

lymphangioleiomyomatosis

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