Radioterapia en mielofibrosis ganglionar refractaria a la terapia dirigida

Abstract

Primary myelofibrosis is a disease of the bone marrow characterized by fibrosis, associated with splenomegaly and extramedullary haematopoiesis. In peripheral blood, a pattern of leucoerythroblastosis and tear drop cells, as well as elevated levels of pro-inflammatory and angiogenic cytokines is observed.

There are two types: primary myelofibrosis, which is not associated with any bone marrow condition, and secondary disease, which is associated with myeloproliferative syndrome conditions such as essential thrombocytosis or polycythaemia vera.

The incidence in the United States ranges from 0.21 to 0.25 cases per 100 thousand inhabitants, it is associated more often with the male gender, and mean age at diagnosis is 67 years.

The treatment of primary myelofibrosis can be in different forms, depending on the goal of treatment, with curative or palliative intent. Treatment modalities include surgical procedures with splenectomy, pharmacological treatment with drugs to produce cell inhibition such as hydroxycarbamide, immunomodulators such as thalidomide, or targeted therapy such as ruxolitinib; radiation therapy is generally used to treat medical treatment-refractory splenomegaly or in those patients with any surgical contraindication and, finally, bone marrow transplantation has proven to be the only treatment that modifies survival.