Atlas of Bone Marrow pathology: Polycythemia Vera (PV)

+ Introduction

+ Support

+ Bone Marrow Pathology

Polycythemia Vera (PV)

Introduction

characterised by an uncontrolled erythrocyte production
3 stages:
- prepolycytemic
- polycytemic
- spent phase (postpolycytemic myelofibrosis)
prognosis: with adequate therapy, median survival time is more than 10 years, approx. 20% of patients develop MDS or AML

Etiology

More than 95% of patients carry the somatic JAK2 V617F mutation.

Clinical signs

Histology

pre-polycytemic and polycytemic stages: bone marrow is hypercellular, panmyelosis with a relative elevation of erythropoiesis (M:E is 1 – 2:1 or even less), clusters of polymorphopus megakaryocytes, no stromal iron, none or only slight fibrosis
postpolycytemic myelofibrosis (spent phase): extensive bone marrow fibrosis with residual hematopoiesis, clusters of dysplastic megakaryocytes, dilatated sinuses with hematopoetic cells, osteosclerosis, extramedular hematopoiesis

Pictures