Info

🌱 來自: primary myelofibrosis (PMF)

diagnostic evaluation of primary myelofibrosis (PMF) 

(Blood 2010;115:1703 & 2016;127:2391)

• Anemia with variable WBC and platelet counts

Peripheral smear

→ “leukoerythroblastic” (teardrop cells, nucleated RBCs, immature WBCs); large abnormal platelets

BM aspirate

→ “dry” tap; BM bx → severe fibrosis, replacement by reticulin & collagen

Mutation

• JAK2 V617F in 45–50%
• CALR mut in 45–50%, 
• MPL mut in 7–10%,
• triple neg in 1–2%

• No BCR-ABL translocation; Pts should not meet criteria for PV or MDS

• DIPSS score for prognosis. High-risk factors: age >65, WBC >25k, Hgb <10, peripheral blasts >1%, symptoms, complex cytogenetics, absence of CALR type 1.