Original ArticleKaposiform Lymphangiomatosis: A Distinct Aggressive Lymphatic Anomaly
Section snippets
Methods
Patients referred to our Vascular Anomalies Center from 1995-2011 with various types of lymphatic anomalies were reviewed at an interdisciplinary conference. Twenty patients had a GLA with novel histopathology. The histologic hallmark was clusters or sheets of “kaposiform” hemosiderotic, spindled lymphatic endothelial cells oriented in parallel fashion amid abnormal and dilated lymphatic channels. These spindled cells were immunoreactive for lymphatic markers (D2-40, Lyve-1, and Prox-1).
Results
All patients had a distinctive histopathologic pattern,10 characteristic imaging, and poor prognosis (Figure 1). They were often referred with a diagnosis of “lymphangiomatosis.” Thoracic disease typically manifested as pleural and/or pericardial effusions. High-resolution computed tomography demonstrated interlobular septal thickening due to dilated lymphatic channels usually with a prominent soft tissue component in the mediastinal, paraspinal, or retroperitoneal region. On magnetic resonance
Discussion
We describe clinical features of a distinct GLA that we term KLA given the foci of “kaposiform” spindled lymphatic endothelial cells and the progressive nature of the anomaly. Intrathoracic disease with worsening respiratory symptoms and hemorrhagic effusions are hallmarks of KLA. Current therapies are largely temporizing.
Spindled cells within anomalous pulmonary lymphatic channels have been noted previously,4, 6, 9 but such lesions have not been designated as a specific entity. It is known
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Supported by American Society of Hematology Trainee Research Grant, the National Institutes of Health's Heart, Lung, and Blood Institute (NIH NHLBI) (T32 HL007574 [to S.C.] and K08 HL089509 [to C.T.]), and Lymphatic Malformation Institute (to C.T.). The authors declare no conflicts of interest.