O. Myeloproliferative Disorders

Question 1

What are the 4 types of Myeloproliferative disorder?

Answer 1

CML
Polycythemia Vera
Essential Thrombocytopenia
Myelofibrosis

Question 2

What is the Myeloproliferative disorder of the RBCs?

Answer 2

PV

Question 3

What is the Myeloproliferative Disorder of platelets?

Answer 3

ET

Question 4

What is the myeloproliferative disorder of granulocytes?

Answer 4

CML

Question 5

What age group do the myeloproliferative disorders generally appear in? Exception?

Answer 5

Middle aged or elderly

CML can sometimes present in children

Question 6

Chronic Myeloproliferative Disorders can progress to what?

Answer 6

Acute Leukemia

Question 7

What cytogenic abnormality is associated with CML? What is the test for it?

Answer 7

t(9:22)
ABL on 9 & BCR on 22
Philadelphia Chromosome = fusion –> tyrosine kinase activity
FISH

Question 8

Clinical presentation of CML?

Answer 8

Some are asymptomatic
Fatigue, weakness, weightloss
Striking leukocytosis, granulocytic hyperplasia with left shift, basophils present

Question 9

What is a LAP (Leukocyte Alkaline Phosphatase) used for?

Answer 9

To differentiate between CML & reaction to infection
Low/ansent in CML
High in infection

Question 10

What are the 3 stages of CML? Survival for each? % Blasts for each?

Answer 10

Chronic = 20% blasts = acute leukemia

Question 11

The blast crisis stage of CML looks exactly like what?

Answer 11

AML

Question 12

Which stage of CML is unresponsive to treatment?

Answer 12

Accelerating phase

Question 13

What is the treatment for CML?

Answer 13

Gleevec/imatinib = tyrosine kinase inhibitor

Question 14

CML is a build up of what?

Answer 14

Pluripotent stem cell that could become myeloid or lymphoid (but mostly becomes myeloid)

Question 15

What physical finding is common with CML?

Answer 15

Splenomegaly

Question 16

What 2 neoplastic disorders have the philedelphia chromosome?

Answer 16

CML

ALL

Question 17

What are the clinical findings for PV?

Answer 17

Elevated Hct
Headache
HTN
Itchy skin
Splenomegaly
Thrombosis and bleeding
Elevated WBC
Elevated platelets

Question 18

What is suggested with clusters of megakaryocytes?

Answer 18

PV

Question 19

What is the treatment for PV?

Answer 19

Phlebotomy or hydroxyurea

Question 20

Differential diagnosis for PV?

Answer 20

Hypoxemia
Smoking
High affinity hemoglobin
High EPO

Question 21

PV is an overproduction of what?

Answer 21

Erthrocytes, granulocytes & megakaryocytes

Question 22

What are the 3 stages of PV and the survival for each?

Answer 22

Chronic = 10-15 years
Spent Phase = 2-3 years
Acute Leukemia =

Question 23

What is the clinical presentation of Essential Thombocythemia?

Answer 23

Thrombocytosis
Some asymptomatic
Bleeding and thrombosis

Question 24

What is suspected with aggregates of platelets?

Answer 24

ET

Question 25

How does the bone marrow look in ET?

Answer 25

Could be hypercellular or normal

Increased megakaryocytes

Question 26

What is Primary Myelofibrosis?

Answer 26

Proliferation of granulocytes, megakaryocytes associated with bone marrow fibrosis (connective tissue) & extramedullary hematopoesis

Question 27

What are the 2 stages of myleofibrosis?

Answer 27

Prefibrotic = proliferative stage. Mild hepatosplenomegaly, mild blood changes
Fibrotic = symptoms

Question 28

What does a positive stain for reticulin suggest?

Answer 28

Myelofibrosis

Question 29

What do tear drop shaped RBCs suggest?

Answer 29

Myelofibrosis

Question 30

What does excessive pink staining on a bone marrow biopsy suggest?

Answer 30

Myelofibrosis

Question 31

What is leukoerythroblastic appearance on a smear?

Answer 31

Nucleated erythroid cells, left shifted myeloid cells, darcocytes

Question 32

What mutation is seen in all of the myeloproliferative disorders accept CML?

Answer 32

Jak2 muation –> constituitively active tyrosine kinase

Question 33

How do the 4 myeloproliferative disorders present initially?

Answer 33

CML‐high WBC, PLT
PV‐high RBC/ Hgb, WBC, PLT
ET‐typically only high PLT
CIMF‐high WBC, PLT

Question 34

How do the 4 myeloproliferative disorders present after a prolonged course?

Answer 34

All CMPD may progress (rarely with ET, invariably with CIMF) to marrow fibrosis and morphologically resemble CIMF, at this stage one cannot reliably determine what it was before; now the peripheral blood counts may be low/ normal

Question 35

What is a myelodysplastic disorder?

Answer 35

Clonal defect in stem cells which lead to defects in cell differentiation & ineffective erythropoesis

Question 36

Key difference between myelodysplastic and myeloproliferative disorders?

Answer 36

myelodysplastic = decreased production of cells
Myeloproliferative = increase in production of cells

Question 37

What are the 2 main types of myelodysplastic syndromes?

Answer 37

Primary = unknown cause, most related to cytogenic abnormalities
Therapy Related = 2-8 years after treatment with radiation or cytotoxic (akylating gents or topoisomerase)

Question 38

What are the clinical presentations of MDS?

Answer 38

Anemia, nuetropenia & thrombocytopenia (+ associated symptoms)
Low retic count
Macrocytic RBCs (High MCV)
Hypercellular marrow

Question 39

What is suggested by psuedo pelger huet cells? What do they look like?

Answer 39

MDS

Bilobed nuclei of nuetrophil = sun glasses

Question 40

Ringed sinderoblasts can be seen with what neoplastic disorder?

Answer 40

MDS

Question 41

Basophils are characteristically elevated in what?

Answer 41

CML

Question 42

What is a common secondary cause of polycythemia?

Answer 42

smoking

Question 43

What system determines the prognosis of MDS?

Answer 43

international prognostic scoring system (IPSS)