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Primary myelofibrosis is a disease that has been characterized by a number of terms in previous literature. The International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) convened in April and November 2006 to clarify definition, response criteria and nomenclature of this condition [1]. The term primary myelofibrosis (PMF) was chosen over several other designations including chronic...
The “classical” myeloproliferative neoplasms such as essential thrombocythemia (ET), polycythemia vera (PV), primary myelofibrosis (PMF) and chronic myeloid leukemia (CML) are relatively rare disorders. Due to the long life span of most patients with these diseases however, the prevalence is quite high, so that patients with these diseases are commonly seen in hematological outpatient departments.
Primary myelodysplastic syndromes (MDS), the most common hematologic malignancy to affect the elderly, are clonal disorders of hematopoietic stem cells. MDS is characterized by an increased but ineffective and dysplastic hematopoiesis as well as peripheral cytopenias. The abnormal hematopoietic clone partly gives rise to mature, but functionally and morphologically abnormal blood cells, and, at least...
Essential Thrombocythemia (ET) is a chronic myeloid disorder with megakaryocytic proliferation in the bone marrow resulting in a persistent increase in platelets in the peripheral blood, with ensuing thrombohemorrhagic symptoms. Furthermore, mild leukocytosis, lack of hepatosplenomegaly and excellent prognosis with only rare transformation to acute leukemia are typical characteristics of this disease.
Chronic myelogenous leukemia (CML) is a chronic myeloproliferative disorder with an uncontrolled production of maturing neutrophils being the predominant laboratory feature. The presence of translocation t(9;22), the so-called Philadelphia chromosome, is commonly acknowledged as the disease initiating event, and as such, is essential for the diagnosis of CML. This chapter addresses the pathophysiology...
The term chronic “myelomonocytic” leukemia (CMML) indicates that all cells of the myeloid lineage are involved (myelo-), but emphasizes the prominence of monocytoid features (“-mono-”). The hallmarks of CMML are peripheral monocytosis >1, 000/μl, with <20% bone marrow blasts and the presence of bone marrowdysplasia.CMML shares clinical and biological features with both myelodysplastic syndromes...