Overview
Definition
Clonal stem cell disorder causing bone marrow fibrosis.
Epidemiology
Varies globally depending on genetics, environment, or exposure.
Etiology & Risk Factors
- JAK2, CALR, or MPL mutations.
Clinical Symptoms
- Massive splenomegaly (extramedullary hematopoiesis)
- severe fatigue
- weight loss.
Clinical Approach
Diagnosis
- Bone marrow biopsy (dry tap
- severe fibrosis)
- Smear (Teardrop RBCs).
Management
- Ruxolitinib (JAK inhibitor)
- Stem cell transplant.
Complications
- Bone marrow failure
- Leukemia transformation.