Myeloproliferative

Question 1

How are myeloproliferative syndromes generally characterized?

Answer 1

Too many blood cells

Question 2

General definition of polycythemia vera (PCV)

Answer 2

Too many RBCs

Question 3

General definition of essential thrombocytosis (ET)

Answer 3

Too many platelets

Question 4

General definition of chronic myelogenous or chronic myeloid leukemia (CML)

Answer 4

Too many neutrophils

Question 5

Define myelofibrosis

Answer 5

Too many RBCs, platelets and neutrophils with prominent immature RBCs

Question 6

Describe myeloproliferative syndromes

Answer 6

• Neoplastic blood cell disorders
• Insidious onset w/vague symptoms
• Splenomegaly
• Cure NOT possible

Question 7

Describe polycythemia vera

Answer 7

Elevated RBC mass a/w a mild increase in WBCs and platelets

Question 8

Etiology of PCV

Answer 8

JAK2 gene mutations in 90% of cases

Question 9

PE findings of PCV

Answer 9

• Ruddy complexion

- Mild splenomegaly

Question 10

Diagnostic tests of PCV

Answer 10

• Total body plasma volume
• Total body RBC volume or mass
• Difficult to obtain in practice

Question 11

Lab findings of PCV

Answer 11

• Normal ABG
• Normal Fe/TIBC
• Normal or slightly high B12
• Normal folate
• Low serum erythropoietin

Question 12

Treatment of PCV

Answer 12

• Decrease Hct via phlebotomy until Fe deficient
• AVOID chemo drugs (Hydrea)
• Anagrelide can be used to decrease platelets
• Ruxolitinib can decrease platelets/splenomegaly but may cause severe anemia (pt must be JAK2 positive to work)

Question 13

What are the lab findings of a patient with well controlled PCV?

Answer 13

• Normal (or near normal) Hct

- Low MCV

Question 14

Why should chemo drugs such as Hydrea be avoided in PCV patients?

Answer 14

Increase the risk of secondary leukemias

Question 15

How can Anagrelide be used to treat PCV?

Answer 15

Decreases platelet count

Question 16

How can Ruxolitinib be used to treat PCV?

Answer 16

• Decreases platelets and splenomegaly
• May cause severe anemia
• Only works if pt is JAK2 positive

Question 17

WHO definition of Essential Thrombocytosis (ET)

Answer 17

Platelet count over 600,000 without other etiologies (Fe deficiency, co-existent infection/inflamm process, bleeding, other myeloproliferative disorder)

Question 18

Clinical presentation of ET

Answer 18

• MC in females
• HA, easy bruising/bleeding
• PE usually normal (mild splenomegaly may be present, but massive splenomegaly should suggest another diagnosis)

Question 19

Lab findings of ET

Answer 19

• Normal Hgb and Hct
• Normal or slightly high WBCs (normal diff)
• Normal Fe/TIBC
• High platelets w/giant plts
• JAK2 V617F present

Question 20

How can elevated platelets affect chem labs of a patient?

Answer 20

Increased K - released from platelets when clot forms in serum tube

Question 21

How can elevated platelets affect ABG results?

Answer 21

Decreased pO2 - due to hypermetabolic state in ABG syringe if not quickly cooled on ice

Question 22

Treatment of ET

Answer 22

• Conservative and symptom based (lower platelets until pt is asymp)
• Anagrelide or Hydrea are mainstays

Question 23

What is CML characterized by?

Answer 23

• High WBC count w/immature blood cells normally seen in BM but now in peripheral blood
• Thrombocytosis and anemia are common
• Basophilia is almost diagnostic

Question 24

What is the most significant factor of CML?

Answer 24

Philadelphia chromosome creating uncontrolled WBC production

Question 25

Lab findings of CML

Answer 25

- Normal ESR - Low leukocyte alkaline phosphatase - Bands, meylocytes on smear - Hypercellular BM w/less than 5% blasts

Question 26

Phases of CML

Answer 26

1. Chronic (modest symptoms) 2. Accelerated 3. Blast Crisis (clinical picture looks like acute leukemia)

Question 27

Treatment of CML

Answer 27

- Refer to hematology - Goal is complete molecular remission - Imatinib (oral TKI, minimal side effects)

Question 28

What is imatinib used to treat?

Answer 28

CML

Question 29

How does imatinib work?

Answer 29

Blocks ATP binding site of ABL kinase

Question 30

Characteristics of myelofibrosis w/myeloid metaplasia (MMM)

Answer 30

- Increased marrow reticulum - Increased marrow fibrosis - Extramedullary hematopoiesis - Splenomegaly

Question 31

Why is early MMM sometimes difficult to distinguish from other myeloproliferative syndromes?

Answer 31

Increased marrow reticulum can be seen in most other myeloproliferative syndromes

Question 32

Classic lab finding of MMM

Answer 32

Leuko-erythroblastic peripheral smear (showing immature RBCs and WBCs)

Question 33

Treatment of MMM

Answer 33

Mainly supportive - Anemia treated w/transfusion - Splenectomy - Hydrea or Ruxolitinib to control hypermetabolic symptoms - BM transplant potentially curative

Question 34

Poor prognosis factors for MMM

Answer 34

- Over 65 yo - WBC over 25,000 - Blasts over 10% - Constitutional symptoms - Hgb less than 10

Question 35

What s/s are NOT seen in myelodysplastic syndromes (MDS)?

Answer 35

Splenomegaly and extramedullary hematopoiesis

Question 36

MDS is characterized by?

Answer 36

- Cytopenias (usually of more than 1 cell line) | - Often a chromosomal abnormality but NOT always

Question 37

What is aplastic anemia characterized by?

Answer 37

Pancytopenia a/w bone marrow hypoplasia

Question 38

Treatment of aplastic anemia

Answer 38

- Supportive until definitive treatment planned by heme - Transfusion - BM transplant potentially curative

Question 39

Describe pure red cell aplasia (PRCA)

Answer 39

- Very rare - Only RBC precursors affected - Etiologies and associated diseases are similar to aplastic anemia - Treatment is immunosuppression, supportive, sometimes anti-virals

Question 40

Who is affected by MDS?

Answer 40

Primarily older adults (over 70) | NOT rare

Question 41

Splenomegaly suggests MDS OR MPD?

Answer 41

MPD

Question 42

Clinical presentation of MDS

Answer 42

- Insidious onset of fatigue - SOB - Possibly angina

Question 43

Typical lab findings of MDS

Answer 43

- Macrocytosis and abnormally formed RBCs/WBCs | - Hypercellular BM w/dysplastic changes

Question 44

Lab finding that indicates better prognosis for MDS

Answer 44

Ringed sideroblasts

Question 45

Lab finding that indicates worse prognosis for MDS

Answer 45

Increased percentage of blasts

Question 46

What does RAEB-2 indicate in MDS?

Answer 46

Poor prognosis - survival less than 6 months

Question 47

Treatment of MDS

Answer 47

- Mainly supportive | - BM transplant for younger patients

Question 48

What type of treatment should be avoided in MPS if possible?

Answer 48

Chemo