Primary splenic involvement in kaposiform lymphangiomatosis: A case report

Kaposiform Lymphangiomatosis (KLA) is a rare, pathologically distinct lymphatic anomaly characterized by abnormal lymphatic channel malformation and a high mortality rate. Previous studies describe primary thoracic involvement.

A previously healthy eighteen-month-old female presented to the pediatric emergency department with sudden onset of petechiae and bruising. Initial CBC demonstrated profound anemia and thrombocytopenia with rapid progression to disseminated intravascular coagulopathy. The child was admitted to the pediatric ICU where hematologic workup included a bone marrow biopsy negative for leukemia and a negative infectious workup. Pediatric Surgery was consulted for splenomegaly with concern for primary splenic process and consumptive coagulopathy. She underwent emergent splenectomy and following operative intervention her clinical status stabilized with rapid resolution of the coagulopathy and thrombocytopenia. Pathologic evaluation of the spleen confirmed the diagnosis of KLA. Subsequent workup failed to reveal any thoracic or mediastinal involvement of KLA.

Although splenic involvement has been described in patients with KLA, according to the published literature, the significant lesions exist in the thoracic cavity. To our knowledge, this is the first reported case of severe consumptive coagulopathy from KLA isolated to the spleen.

Although rare, KLA should be considered early in the diagnosis of a patient presenting with sudden onset of profound thrombocytopenia and coagulopathy.