Myeloproliferative Neoplasms

Hematology Week 2

A group of disorders that cause a malignant increase of mature RBC, PLT, and granulocytes due to HSC mutations

Myeloproliferative neoplasms

What cancers may result from chronic MPN?

AML (most likely) or ALL (25% of cases)

What are the four WHO classified MPN?

Chronic myeloid leukemia (CML)

Polycythemia vera (PV)

Essential thrombocythemia (ET)

Primary myelofibrosis (PMF)

The Philadelphia chromosome is seen in what MPN?

CML

The JAK2 mutation is seen in what MPN?

PV, ET, and PMF

What cells are seen in CML?

Neutrophilia (with left shift), basophilia, eosinophilia

What genes result in Philadelphia chromosome?

Translocation of BCR and ABL1

What is the placement of the BCR gene?

Chromosome 22, position 11, protein 160

What is the placement of the ABL1 gene?

Chromosome 9, position 34, protein 125

What protein is created by the formation of Philadelphia chromosome?

p210

What does the ABL1 p125 usually synthesize for?

Tyrosine kinase

What does BCR p160 usually synthesize for?

Serine and threonine kinase

What effect does BCR-ABL1 have in the signal transduction pathway?

Increase in myeloid proliferation

Decrease in differentiation

Decrease in adhesion to BM

Decrease in apoptosis

What does a LAP screen for?

Differentiates between a leukamoid reaction due to infection or CML

A high LAP score and a low LAP score reflects which conditions?

High LAP: leukemoid reaction

Low LAP: CML