11-19 L2 MPN & MDS

Question 1

MPN

Myeloproliferative neoplasms

Answer 1

• excessive accumulation of mature forms with relatviely normal function

Question 2

Define the 4 subtypes of MPN

Answer 2

• Polcytehmia vera
• Chronic myelogenous leukemia
• Essential Thrombocythemia
• Primary myelofibrosis

Question 3

CML blast crisis

Answer 3

• usually AML but can be ALL
• Occurs with development of new cytogenetic abnormalities
• accelerated or blast phase patients show resistance to tyrosine kinase inhibitors
• Duplication of Philidelphia chormosome, trisomy 8 or 19

Question 4

Myelofibrosis

• ‘burnt out MPN’*

Answer 4

• occurs in other MPN after many years of disease
• Fibrosis is a space occuyping marrow lesion
  
  • Leukoerhroblastosis
  • Teardrop red cells
  • Circulating nucleated red cells
  • Left-shifted granulocytes

Question 5

MDS

Myelodysplastic Syndrome

name one key feature

Answer 5

excessive accumulation of dysfunctional cells in the bone marrow with peripheral cytopenias

• Ring sideroblasts (abnormal utlization of iron by erythoblasts)

Question 6

examples of MDS

Answer 6

• Granulocytic dysplasia
• Erthryoid dysplasia
• Megakaryocytic dysplasia
  *