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Myelofibrosis was first described in 1879 by Gustav Heuck. [33] [34] Eponyms for the disease are Heuck-Assmann disease or Assmann's Disease, for Herbert Assmann, [35] who published a description under the term "osteosclerosis" in 1907. [36] It was characterised as a myeloproliferative condition in 1951 by William Dameshek. [37] [38]
Myeloproliferative neoplasms (MPNs) are a group of rare blood cancers in which excess red blood cells, white blood cells or platelets are produced in the bone marrow. Myelo refers to the bone marrow, proliferative describes the rapid growth of blood cells and neoplasm describes that growth as abnormal and uncontrolled.
Bone biopsy shows abnormal megakaryocytes, macrocytic erythropoiesis, and defects in neutrophil production and fibrosis of the marrow (myelofibrosis). Clinically, patients present with reduction in the count of all blood cells (pancytopenia), very few blasts in the peripheral blood, and no or little spleen enlargement (splenomegaly).
One study found the median age at diagnosis to be 60 years, [9] while a Mayo Clinic study in Olmsted County, Minnesota found that the highest incidence was in people aged 70–79 years. [40] The overall incidence in the Minnesota population was 1.9 per 100,000 person-years, and the disease was more common in men than women. [ 40 ]
Myelophthisic anemia (or myelophthisis) is a severe type of anemia found in some people with diseases that affect the bone marrow. Myelophthisis refers to the displacement of hemopoietic bone-marrow tissue [1] by fibrosis, tumors, or granulomas. The word comes from the roots myelo-, which refers to bone marrow, and phthisis, shrinkage or atrophy.
Ruxolitinib (sold under the brand names Jakafi and Jakavi among others, and as Opzelura in cream form) is a medication used for the treatment of intermediate or high-risk myelofibrosis, [6] a type of myeloproliferative neoplasm that affects the bone marrow; [11] [12] polycythemia vera, when there has been an inadequate response to or intolerance of hydroxyurea; [6] [13] and steroid-refractory ...
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Hydroxyurea treated patients had a lower incidence of arterial thrombosis, lower incidence of severe bleeding and lower incidence of transformation to myelofibrosis, but the risk of venous thrombosis was higher with hydroxycarbamide than with anagrelide. It is unknown whether the results are applicable to all ET patients.