[Idiopathic myelofibrosis with cutaneous extramedullary hematopoiesis]

Abstract

Idiopathic myelofibrosis is a chronic myeloproliferative syndrome characterized by the proliferation of the three hemopoietic series and the marrow connective tissue and the development of extramedullary hemopoiesis in the liver and spleen. Cutaneous extramedullary hemopoiesis is and uncommon finding, and only thirteen cases have been reported up to now. We report the first case in the Spanish literature. The patient was a 78-year-old male in whom skin lesions preceded the diagnosis of myelofibrosis. These lesions, consisting of violet coloured maculae and papulae, contained elements from the three hemopoietic series. Therapy with hydroxyurea was started, but the patient died because of cardiorespiratory complications.