Case Study Discuss the pathpyysiology of Myelofibrosis Essay

Case Study Discuss the pathpyysiology of Myelofibrosis - Essay physical exertionThis disease is characterized by fibrosis and sclerosis of study marrow. To compensate for the hematopoietic prison cell population, extramedullary hematopoiesis takes place in the spleen, accounting for the massive enlargement of the spleen. This disease occurs predominantly in subjects over the age of 54 years. The bone marrow fibrosis is the hallmark of pathological findings in such(prenominal) cases, where extensive fibrosis with resultant peripheral blood leukoerythroblastosis is expect as a typical histopathologic picture on bone marrow biopsy (Tefferi, 2000, p. 1255-1265).This clinically heterogeneous collection of diseases stems from clonal proliferation of stem cell origin and is characterized at least initially by marrow hypercellularity with variable degrees of marrow fibrosis and an increase in the production of one or more terminally differentiated cell types. These differentiated elemen ts may accumulate in the bone marrow, in the peripheral blood, and in other organs, such as, spleen. All these diseases demonstrate a variable tendency to undergo disease progression that may revoke in bone marrow failure or in transformation to an acute phase malignant disease. The evaluation of bone marrow histology holds an important role in defining the pathology of this disease, by mainly judgment out unsuspected pathology. The pathologic changes are subtle until the disease has progressed, and therefore, classification of these upsets benefits from the integration of the morphologic de coloreds with clinical, hematologic, and cytogenetic findings. Of major importance is the presence or absence of Philadelphia chromosomes (BCR/ABL or translocation922). This group of diseases constitutes the classical group of BCR/ABL-negative degenerative myeloproliferative disorders. The disease is regarded as one of the chronic myeloproliferative disorders. Recently considerable progres s has been made in sense its pathogenesis, although this has yet to result in significant therapeutic advances. Indeed, its prognosis remains poor when compared to other BCR-ABL-negative chronic myeloproliferative disorders with death resulting from cardiac failure, infection, hemorrhage, and leukemic transformation (Barosi, 2003, p. 1211-1226).Hemapoetic Components It has been appreciated for many years that MF is a clonal disorder and that the disease arises from the proliferation of malignant pluripotential stem cells. Recently, using fluorescent in situ hybridization (FISH), there is evidence that both(prenominal) B and T cells can be involved, while karyotypic analysis has shown that the stromal proliferation is polyclonal, or reactive, and not part of the rudimentary clonal hematopoiesis. An increased number of circulating hematopoietic precursors, including pluripotent and lineage restricted progenitor cells is a feature of MF and is likely to result from the proteolytic r elease of stem cells from the marrow. It is also possible that the spleen and liver contribute to the circulating progenitor pool as splenectomy temporarily normalizes levels. The high level of circulating progenitor cells is reflected in the significantly increased peripheral