@entity4018 with @entity4018 is a @entity1503 currently classified with @entity6582 vera and essential @entity1148 as a @entity102 . The median age at diagnosis is 60 years, and more than 90% of @entity1 are diagnosed after age 40 years. Clinical manifestations include massive @entity381 , progressive @entity417 , profound constitutional symptoms, and extramedullary hematopoiesis. The diagnosis is confirmed by bone marrow examination after other causes of @entity4018 are ruled out. Median survival is 5 years and causes of @entity204 include @entity1778 . Prognosis is adversely affected by the presence of @entity417 (hemoglobin <10 g/dl), @entity1431 or @entity491 (white blood cells >30,000/ micro l), circulating blasts, and @entity11744 symptoms. Conventional treatment is palliative and does not improve survival. In this regard, @entity342 preparations, corticosteroids, and @entity1307 are useful for the treatment of disease-associated @entity417 . Symptomatic @entity381 is best managed by cytoreductive therapy or surgical removal. Radiation therapy is most useful in the treatment of @entity1210 . New treatment approaches include the use of @entity2816 alone or in combination with @entity229 and hematopoietic stem cell transplantation.

The forgotten myeloproliferative disorder: XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity6582 :: ('MESH:D011086', 'Disease') :: ['polycythemia'] @entity1148 :: ('MESH:D013922', 'Disease') :: ['thrombocythemia'] @entity417 :: ('MESH:D000740', 'Disease') :: ['anemia'] @entity229 :: ('MESH:D011241', 'Chemical') :: ['prednisone'] @entity11744 :: ('MESH:C565476', 'Disease') :: ['hypercatabolic'] @entity2816 :: ('MESH:D013792', 'Chemical') :: ['thalidomide'] @entity1307 :: ('2056', 'Gene') :: ['erythropoietin'] @entity102 :: ('MESH:D002908', 'Disease') :: ['chronic myeloproliferative disease'] @entity1503 :: ('MESH:D006402', 'Disease') :: ['hematologic disorder'] @entity1778 :: ('MESH:D007938', 'Disease') :: ['leukemic transformation'] @entity4018 :: ('MESH:D055728', 'Disease') :: ['Myelofibrosis', 'myeloid metaplasia', 'myelofibrosis'] @entity381 :: ('MESH:D013163', 'Disease') :: ['splenomegaly'] @entity1431 :: ('MESH:D007970', 'Disease') :: ['leukopenia'] @entity342 :: ('CHEBI:50113', 'Chemical') :: ['androgen'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity491 :: ('MESH:D007964', 'Disease') :: ['leukocytosis'] @entity1210 :: ('MESH:C536227', 'Disease') :: ['nonhepatosplenic extramedullary hematopoiesis']
@entity4018 :: (MESH:D055728,Disease) :: ['myeloid metaplasia']
