Primary myelofibrosis

Primary myelofibrosis
Updated: 7/15/2019
© Jun Wang, MD, PhD

General features
  • Clonal neoplastic transformation of hematopoietic stem cells
  • Fibrosis caused by activation of background reactive fibroblasts
  • More common in Caucasians, Ashkenazi Jews
Clinical presentations
  • Anemia with teardrop-shaped RBCs
  • Extramedullary hematopoiesis
  • Leukoerythroblastosis: Nucleated erythroid and early granulocyte progenitors in peripheral blood, due to premature release
  • Marked hepatosplenomegaly
  • Dry tap: Failure to obtain bone marrow on attempted marrow aspiration
Key pathogenesis
  • Megakaryocytes production of TGF-β, PDGF and bFGF
  • Growth factors induce fibrosis and angiogenesis
Key Laboratory findings
Key morphological features
Genetic abnormalities
  • Activation of JAK2, CALR, MPL
Differential diagnosis


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Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4. Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas Multiple endocrine neoplasms 14. B
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