JSH guideline for tumors of hematopoietic and lymphoid tissues—lukemia: 4. Chronic myelogenous leukemia (CML)/myeloproliferative neoplasms (MPN)

Excerpt

Myeloproliferative neoplasms (MPN), a group of diseases that develop through neoplastic transformation at the level of hematopoietic stem cells, are characterized by marked proliferation of myeloid cells (i.e., granulocytes, erythroblasts, megakaryocytes, and mast cells) [1]. The category of MPN includes chronic myelogenous leukemia (CML), chronic neutrophilic leukemia (CNL), polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia (CEL), hypereosinophilic syndrome (HES), mastocytosis, and myeloproliferative neoplasms, unclassifiable (MPN, U). Early stage MPN exhibit hyperplasia of bone-marrow cells with capacity for differentiation and increased peripheral granulocytes, red blood cells (RBCs), and platelets. Physical findings include splenomegaly and hepatomegaly. MPN produce few subjective symptoms at onset, but progress in stages along with general symptoms. They ultimately progress to bone-marrow failure, the fatal terminal stage, caused by myelofibrosis or loss of maturation potential through transformation (blast crisis). A different treatment approach is used for CML from those for the other types of MPN. These guidelines primarily cover treatments for CML, PV, ET, and PMF. …