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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]
Pacritinib, sold under the brand name Vonjo, is an anti-cancer medication used to treat myelofibrosis. [1] [2] It is a macrocyclic protein kinase inhibitor. It mainly inhibits Janus kinase 2 (JAK2) and Fms-like tyrosine kinase 3\CD135 (FLT3). Common side effects include diarrhea, low platelet counts, nausea, anemia, and swelling in legs. [2]
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]
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).
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.
Other pre-existing bone-marrow disorders such as acquired aplastic anemia following immunosuppressive treatment and Fanconi anemia can evolve into MDS. [15] MDS is thought to arise from mutations in the multipotent bone-marrow stem cell, but the specific defects responsible for these diseases remain poorly understood.
Acute panmyelosis with myelofibrosis: C94.4: Acute panmyelosis with myelofibrosis (APMF) is a poorly defined disorder that arises as either a clonal disorder, or following toxic exposure to the bone marrow. Lymphosarcoma cell leukemia: Acute leukaemia of unspecified cell type: Blastic phase chronic myelogenous leukemia
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.