"Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly associated with a poor prognosis. Disease hallmarks include multifocal, intra- and extra-thoracic lymphatic malformations, thrombocytopenia and consumptive coagulopathy."
"Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly associated with a poor prognosis. Disease hallmarks include multifocal, intra- and extra-thoracic lymphatic malformations, thrombocytopenia and consumptive coagulopathy."
"organomegaly, especially splenomegaly, hepatomegaly or nephromegaly"
"retroperitoneal involvement is common and may demonstrate an enhancing, infiltrative soft tissue mass, commonly with extension to other structures, including the mesentery, hepatic or renal hila, or along vessels in these regions 4"
"There is no consensus on treatment, but patients are treated with a combination of medical and surgical therapies. Medical therapies may include combinations of steroids, chemotherapy and immunomodulators (interferon, sirolimus, vincristine) but responses are unpredictable. Surgical procedures are usually for symptomatic benefit; however, splenectomy appears beneficial in some patients for refractory thrombocytopenia."
"The exact prevalence and incidence of kaposiform lymphangiomatosis is unknown, but the disease is very rare. The disease usually presents at birth or early childhood; however, reports exist of disease manifestations later in life 1. Males and females appear equally affected. The exact cause is unknown; however, genetic factors and changes in utero are thought to contribute to disease development."
"The exact prevalence and incidence of kaposiform lymphangiomatosis is unknown, but the disease is very rare. The disease usually presents at birth or early childhood; however, reports exist of disease manifestations later in life 1. Males and females appear equally affected. The exact cause is unknown; however, genetic factors and changes in utero are thought to contribute to disease development."
"There is no consensus on treatment, but patients are treated with a combination of medical and surgical therapies. Medical therapies may include combinations of steroids, chemotherapy and immunomodulators (interferon, sirolimus, vincristine) but responses are unpredictable. Surgical procedures are usually for symptomatic benefit; however, splenectomy appears beneficial in some patients for refractory thrombocytopenia."
"Histopathology generally shows malformed dilated lymphatic channels associated with clusters or sheets of spindle-like cells. There is no atypia or dysplasia. Immunochemical staining is positive for markers of lymphatic endothelium."
"There is no consensus on treatment, but patients are treated with a combination of medical and surgical therapies. Medical therapies may include combinations of steroids, chemotherapy and immunomodulators (interferon, sirolimus, vincristine) but responses are unpredictable. Surgical procedures are usually for symptomatic benefit; however, splenectomy appears beneficial in some patients for refractory thrombocytopenia."