Atlas of Bone Marrow pathology: Primary Myelofibrosis (PMF)

+ Introduction

+ Support

+ Bone Marrow Pathology

Primary Myelofibrosis (PMF)

Clinical signs

proliferation, especially of the magakaryocytic and granulocytic lineages, associated with fibrosis
2 stages:
- pre-fibrotic/cellular
- fibrotic
early symptoms are non-secific, in later stages symptoms include anemia, organomegaly, subfebrile temperatures, fatigue, weight loss
prognosis: median survival time is approx. 3 – 7 years for patients diagnosed in the fibrotic stage and approx. 10 – 15 years for patients diagnosed during the early pre-fibrotic stage, 5 – 30% of patients develop AML

Histology

pre-fibrotic stage: hypercellular bone marrow, clusters of increased numbers of large megakaryocytes with hyperlobated nuclei, dysplastic megakaryocytes, a relative increase in granulopoiesis (M : E ratio is approx. 5 : 1), none or only slight fibrosis (MF-0 or MF-1)
fibrotic stage: extensive fibrosis of the bone marrow (MF-2 nebo MF-3) with residual hematopoiesis, clusters of dysplastic megakaryocytes, dilatated sinuses with hematopoietic cells, osteosclerosis, extramedullar hematopoiesis

Pictures