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Myelofibrosis
B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Myelosclerosis
          • Definitions

            • Chronic myeloproliferative disorder → marrow fibrosis
            • Most cases are secondary to other processes
              • Leukemia (myelofibrosis may be diagnosed either prior to or at time of diagnosis of leukemia)
              • Non-Hodgkin lymphoma and Hodgkin disease
              • Essential thrombocythemia
              • Nonmalignant causes (including infections, renal osteodystrophy, systemic lupus erythematosus, juvenile idiopathic arthritis, pernicious anemia, Gaucher disease, exposure to radiation, toxins)
            • Primary myelofibrosis: 2 classes
              • Agnogenic myeloid metaplasia with myelofibrosis: Indolent myeloproliferative syndrome (usually has splenomegaly)
              • Acute myelofibrosis (usually no splenomegaly)

          IMAGING

          • General Features

            • Imaging Recommendations

              • Radiographic Findings

                • MR Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Kröger N et al: Impact of allogeneic stem cell transplantation on survival of patients less than 65 years with primary myelofibrosis. Blood. 125(21):3347-50, 2015
                                  2. Markiewicz M et al: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis. Eur J Haematol. ePub, 2015
                                  3. Palandri F et al: Mutations and long-term outcome of 217 young patients with essential thrombocythemia or early primary myelofibrosis. Leukemia. 29(6):1344-9, 2015
                                  4. Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
                                  5. Kvasnicka HM et al: Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 85(1):62-9, 2010
                                  6. Etienne A et al: Myelofibrosis-associated lymphoproliferative disease: retrospective study of 16 cases and literature review. Adv Hematol. 2009:179847, 2009
                                  7. Mesa RA et al: Emerging drugs for the therapy of primary and post essential thrombocythemia, post polycythemia vera myelofibrosis. Expert Opin Emerg Drugs. 14(3):471-9, 2009
                                  8. Orazi A et al: Myelodysplastic syndromes. Am J Clin Pathol. 132(2):290-305, 2009
                                  9. Plo I et al: Molecular and genetic bases of myeloproliferative disorders: questions and perspectives. Clin Lymphoma Myeloma. 9 Suppl 3:S329-39, 2009
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Diagnostic Checklist

                                          TERMINOLOGY

                                          • Synonyms

                                            • Myelosclerosis
                                          • Definitions

                                            • Chronic myeloproliferative disorder → marrow fibrosis
                                            • Most cases are secondary to other processes
                                              • Leukemia (myelofibrosis may be diagnosed either prior to or at time of diagnosis of leukemia)
                                              • Non-Hodgkin lymphoma and Hodgkin disease
                                              • Essential thrombocythemia
                                              • Nonmalignant causes (including infections, renal osteodystrophy, systemic lupus erythematosus, juvenile idiopathic arthritis, pernicious anemia, Gaucher disease, exposure to radiation, toxins)
                                            • Primary myelofibrosis: 2 classes
                                              • Agnogenic myeloid metaplasia with myelofibrosis: Indolent myeloproliferative syndrome (usually has splenomegaly)
                                              • Acute myelofibrosis (usually no splenomegaly)

                                          IMAGING

                                          • General Features

                                            • Imaging Recommendations

                                              • Radiographic Findings

                                                • MR Findings

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Microscopic Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Kröger N et al: Impact of allogeneic stem cell transplantation on survival of patients less than 65 years with primary myelofibrosis. Blood. 125(21):3347-50, 2015
                                                                  2. Markiewicz M et al: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis. Eur J Haematol. ePub, 2015
                                                                  3. Palandri F et al: Mutations and long-term outcome of 217 young patients with essential thrombocythemia or early primary myelofibrosis. Leukemia. 29(6):1344-9, 2015
                                                                  4. Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
                                                                  5. Kvasnicka HM et al: Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 85(1):62-9, 2010
                                                                  6. Etienne A et al: Myelofibrosis-associated lymphoproliferative disease: retrospective study of 16 cases and literature review. Adv Hematol. 2009:179847, 2009
                                                                  7. Mesa RA et al: Emerging drugs for the therapy of primary and post essential thrombocythemia, post polycythemia vera myelofibrosis. Expert Opin Emerg Drugs. 14(3):471-9, 2009
                                                                  8. Orazi A et al: Myelodysplastic syndromes. Am J Clin Pathol. 132(2):290-305, 2009
                                                                  9. Plo I et al: Molecular and genetic bases of myeloproliferative disorders: questions and perspectives. Clin Lymphoma Myeloma. 9 Suppl 3:S329-39, 2009