tailieunhanh - Chapter 103. Polycythemia Vera and Other Myeloproliferative Diseases (Part 8)

Chronic Idiopathic Myelofibrosis: Treatment No specific therapy exists for chronic IMF. Anemia may be due to gastrointestinal blood loss and exacerbated by folic acid deficiency, and in rare instances, pyridoxine therapy has been effective. However, anemia is more often due to ineffective erythropoiesis uncompensated by extramedullary hematopoiesis in the spleen and liver. Neither recombinant erythropoietin nor androgens, such as Danazol, have proved consistently effective as therapy for anemia. Erythropoietin may worsen splenomegaly and will be ineffective if the serum erythropoietin level is 125 mU/L. A red cell splenic sequestration study can establish the presence of hypersplenism, for which splenectomy is. | Chapter 103. Polycythemia Vera and Other Myeloproliferative Diseases Part 8 Chronic Idiopathic Myelofibrosis Treatment No specific therapy exists for chronic IMF. Anemia may be due to gastrointestinal blood loss and exacerbated by folic acid deficiency and in rare instances pyridoxine therapy has been effective. However anemia is more often due to ineffective erythropoiesis uncompensated by extramedullary hematopoiesis in the spleen and liver. Neither recombinant erythropoietin nor androgens such as Danazol have proved consistently effective as therapy for anemia. Erythropoietin may worsen splenomegaly and will be ineffective if the serum erythropoietin level is 125 mU L. A red cell splenic sequestration study can establish the presence of hypersplenism for which splenectomy is indicated. Splenectomy may also be necessary if splenomegaly impairs alimentation and should be performed before cachexia sets in. In this situation splenectomy should not be avoided because of concern over rebound thrombocytosis loss of hematopoietic capacity or compensatory hepatomegaly. However for unexplained reasons splenectomy increases the risk of blastic transformation. Splenic irradiation is at best temporarily palliative and associated with a significant risk of neutropenia and infection. Allopurinol can control significant hyperuricemia and hydroxyurea has proved useful for controlling organomegaly in some patients. The role of IFN-a is still undefined and its side effects are more pronounced in the older individuals who are usually afflicted with this disorder. Glucocorticoids have been used to control constitutional symptoms and autoimmune complications and may ameliorate anemia alone or in combination with low dose thalidomide 50-100 mg d . Allogeneic bone marrow transplantation is the only curative treatment and should be considered in younger patients reduced-intensity conditioning regimens may permit hematopoietic cell transplantation to be extended to older individuals. .