TAILIEUCHUNG - Chapter 103. Polycythemia Vera and Other Myeloproliferative Diseases (Part 5)

Chronic Idiopathic Myelofibrosis Chronic IMF (other designations include agnogenic myeloid metaplasia or myelofibrosis with myeloid metaplasia) is a clonal disorder of a multipotent hematopoietic progenitor cell of unknown etiology characterized by marrow fibrosis, extramedullary hematopoiesis, and splenomegaly. Chronic IMF is the least common chronic myeloproliferative disorder, and establishing this diagnosis in the absence of a specific clonal marker is difficult because myelofibrosis and splenomegaly are also features of both PV and CML. Furthermore, myelofibrosis and splenomegaly also occur in a variety of benign and malignant disorders (Table 103-3), many of which are amenable to specific therapies not effective in chronic. | Chapter 103. Polycythemia Vera and Other Myeloproliferative Diseases Part 5 Chronic Idiopathic Myelofibrosis Chronic IMF other designations include agnogenic myeloid metaplasia or myelofibrosis with myeloid metaplasia is a clonal disorder of a multipotent hematopoietic progenitor cell of unknown etiology characterized by marrow fibrosis extramedullary hematopoiesis and splenomegaly. Chronic IMF is the least common chronic myeloproliferative disorder and establishing this diagnosis in the absence of a specific clonal marker is difficult because myelofibrosis and splenomegaly are also features of both PV and CML. Furthermore myelofibrosis and splenomegaly also occur in a variety of benign and malignant disorders Table 103-3 many of which are amenable to specific therapies not effective in chronic IMF. In contrast to the other chronic myeloproliferative disorders and so-called acute or malignant myelofibrosis which can occur at any age chronic IMF primarily afflicts individuals in their sixth decade or later. Table 103-3 Disorders Causing Myelofibrosis Malignant Acute leukemia lymphocytic myelogenous megakaryocytic Chronic myelogenous leukemia Hairy cell leukemia Hodgkin disease Idiopathic myelofibrosis Lymphoma Multiple myeloma Myelodysplasia Polycythemia vera Systemic mastocytosis Nonmalignant HIV infection Hyperparathyroidism Renal osteodystrophy Systemic lupus erythematosus Tuberculosis Vitamin D deficiency Thorium dioxide exposure Gray platelet syndrome Etiology The etiology of chronic IMF is unknown. Nonrandom chromosome abnormalities such as 9p 20q- 13q- trisomy 8 or 9 or partial trisomy 1q are common but no cytogenetic abnormality specific to the disease has been identified. The degree of myelofibrosis and the extent of extramedullary hematopoiesis are also not related. Fibrosis in this disorder is associated with overproduction of transforming growth factor 0and tissue inhibitors of metalloproteinases while osteosclerosis is associated with overproduction of

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