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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)
- Clonal hematopoietic BCR::ABL1-negative MPN characterized by
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
- Tefferi A: Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 96(1):145-62, 2021
- Venugopal S et al: Current clinical investigations in myelofibrosis. Hematol Oncol Clin North Am. 35(2):353-73, 2021
- Oon SF et al: Primary myelofibrosis: spectrum of imaging features and disease-related complications. Insights Imaging. 10(1):71, 2019
- O'Sullivan JM et al: Myelofibrosis: clinicopathologic features, prognosis, and management. Clin Adv Hematol Oncol. 16(2):121-31, 2018
- 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
- Markiewicz M et al: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis. Eur J Haematol. 96(3):222-8, 2015
- 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
- Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
- Kvasnicka HM et al: Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 85(1):62-9, 2010
- Etienne A et al: Myelofibrosis-associated lymphoproliferative disease: retrospective study of 16 cases and literature review. Adv Hematol. 2009:179847, 2009
- 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
- Orazi A et al: Myelodysplastic syndromes. Am J Clin Pathol. 132(2):290-305, 2009
- Plo I et al: Molecular and genetic bases of myeloproliferative disorders: questions and perspectives. Clin Lymphoma Myeloma. 9 Suppl 3:S329-39, 2009
- Guermazi A et al: Imaging findings in patients with myelofibrosis. Eur Radiol. 9(7):1366-75, 1999
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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)
- Clonal hematopoietic BCR::ABL1-negative MPN characterized by
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
- Tefferi A: Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 96(1):145-62, 2021
- Venugopal S et al: Current clinical investigations in myelofibrosis. Hematol Oncol Clin North Am. 35(2):353-73, 2021
- Oon SF et al: Primary myelofibrosis: spectrum of imaging features and disease-related complications. Insights Imaging. 10(1):71, 2019
- O'Sullivan JM et al: Myelofibrosis: clinicopathologic features, prognosis, and management. Clin Adv Hematol Oncol. 16(2):121-31, 2018
- 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
- Markiewicz M et al: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis. Eur J Haematol. 96(3):222-8, 2015
- 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
- Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
- Kvasnicka HM et al: Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 85(1):62-9, 2010
- Etienne A et al: Myelofibrosis-associated lymphoproliferative disease: retrospective study of 16 cases and literature review. Adv Hematol. 2009:179847, 2009
- 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
- Orazi A et al: Myelodysplastic syndromes. Am J Clin Pathol. 132(2):290-305, 2009
- Plo I et al: Molecular and genetic bases of myeloproliferative disorders: questions and perspectives. Clin Lymphoma Myeloma. 9 Suppl 3:S329-39, 2009
- Guermazi A et al: Imaging findings in patients with myelofibrosis. Eur Radiol. 9(7):1366-75, 1999
