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Myelofibrosis
Donna G. Blankenbaker, MD, FACR; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Myelosclerosis
            • Myeloproliferative neoplasm (MPN)
          • Definitions

            • Clonal hematopoietic BCR::ABL1-negative MPN characterized by
              • Constitutional symptoms
              • Extramedullary hematopoiesis (splenomegaly, bone marrow fibrosis, and megakaryocytic hyperplasia)
            • 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 displays 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

                                • Clinical Care Points

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Tefferi A: Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 96(1):145-62, 2021
                                    2. Venugopal S et al: Current clinical investigations in myelofibrosis. Hematol Oncol Clin North Am. 35(2):353-73, 2021
                                    3. Oon SF et al: Primary myelofibrosis: spectrum of imaging features and disease-related complications. Insights Imaging. 10(1):71, 2019
                                    4. O'Sullivan JM et al: Myelofibrosis: clinicopathologic features, prognosis, and management. Clin Adv Hematol Oncol. 16(2):121-31, 2018
                                    5. 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
                                    6. Markiewicz M et al: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis. Eur J Haematol. 96(3):222-8, 2015
                                    7. 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
                                    8. Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
                                    9. Kvasnicka HM et al: Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 85(1):62-9, 2010
                                    10. Etienne A et al: Myelofibrosis-associated lymphoproliferative disease: retrospective study of 16 cases and literature review. Adv Hematol. 2009:179847, 2009
                                    11. 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
                                    12. Orazi A et al: Myelodysplastic syndromes. Am J Clin Pathol. 132(2):290-305, 2009
                                    13. Plo I et al: Molecular and genetic bases of myeloproliferative disorders: questions and perspectives. Clin Lymphoma Myeloma. 9 Suppl 3:S329-39, 2009
                                    14. Guermazi A et al: Imaging findings in patients with myelofibrosis. Eur Radiol. 9(7):1366-75, 1999
                                    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
                                              • Myeloproliferative neoplasm (MPN)
                                            • Definitions

                                              • Clonal hematopoietic BCR::ABL1-negative MPN characterized by
                                                • Constitutional symptoms
                                                • Extramedullary hematopoiesis (splenomegaly, bone marrow fibrosis, and megakaryocytic hyperplasia)
                                              • 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 displays 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

                                                                  • Clinical Care Points

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Tefferi A: Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 96(1):145-62, 2021
                                                                      2. Venugopal S et al: Current clinical investigations in myelofibrosis. Hematol Oncol Clin North Am. 35(2):353-73, 2021
                                                                      3. Oon SF et al: Primary myelofibrosis: spectrum of imaging features and disease-related complications. Insights Imaging. 10(1):71, 2019
                                                                      4. O'Sullivan JM et al: Myelofibrosis: clinicopathologic features, prognosis, and management. Clin Adv Hematol Oncol. 16(2):121-31, 2018
                                                                      5. 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
                                                                      6. Markiewicz M et al: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis. Eur J Haematol. 96(3):222-8, 2015
                                                                      7. 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
                                                                      8. Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
                                                                      9. Kvasnicka HM et al: Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 85(1):62-9, 2010
                                                                      10. Etienne A et al: Myelofibrosis-associated lymphoproliferative disease: retrospective study of 16 cases and literature review. Adv Hematol. 2009:179847, 2009
                                                                      11. 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
                                                                      12. Orazi A et al: Myelodysplastic syndromes. Am J Clin Pathol. 132(2):290-305, 2009
                                                                      13. Plo I et al: Molecular and genetic bases of myeloproliferative disorders: questions and perspectives. Clin Lymphoma Myeloma. 9 Suppl 3:S329-39, 2009
                                                                      14. Guermazi A et al: Imaging findings in patients with myelofibrosis. Eur Radiol. 9(7):1366-75, 1999