Multifocal lymphangioendotheliomatosis with thrombocytopenia/cutaneovisceral angiomatosis with thrombocytopenia

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT)/cutaneovisceral angiomatosis thrombocytopenia, first reported as a new nosological entity in 2004, is an extremely rare vascular pathology of the neonatal period without hereditary nature. This disease has poor prognosis. According to previously published data, this characterized by involvement skin and internal organs pathological process. Lesions gastrointestinal tract are almost obligatory disease. MLT associated high mortality, which, according some authors, can reach more than 50%. Fulminant bleedings due specific lesions intestinal mucosa, severe consumption coagulopathy, leading cause death these patients. Currently, there no standard treatment options for MLT. The data about efficacy glucocorticosteroids, vincristine, thalidomide, propranolol, rapamycin MLT, controversial. Here we present case report patient classic clinical spectrum We on polychemotherapeutic regimen liposomal doxorubicin disease: achieved complete hematological response after 2 months from start. A durable was documented 6 treatment. represents challenges, both diagnostic therapeutic point view. Further studies required find optimal regimens condition. patient's parents have consented use de-identified information photos scientific research publications.