Myeloproliferative Myelodysplastic Syndromes

Question 1

myeloproliferative and myelodysplastic syndromes deal with

Answer 1

disorders of clonal stem lines of all three linages platelet, WBC, and RBC are affected

Question 2

three types of myeloproliferative syndromes

Answer 2

1. polycythemia vera
2. essential thrombosis
3. myelofibrosis

Question 3

erythrocytosis

Answer 3

increased RBC volume, increased hemoglobin and hematocrit

Question 4

polycythemia

Answer 4

synonym for erythorocytosis

Question 5

Secondary factors can cause erythrocytosis

Answer 5

these condition tend to lead to erythrocytosis because tissue hypoxia induces EPO to be secrete form the kidneys

1. OSA
2. obesity causing hyperventilation
3. high attitudes
4. high affinity hemoglobin
5. cardiopulmonary disorders
6. high carboxyhemoglobin levels
7. tumor
8. treatment with androgens or EPO
9. rare genetic disorders

Question 6

erythrocytosis can lead to _______

Answer 6

hyperviscosity

Question 7

hyperviscosity symptoms

Answer 7

thickening of the blood; headache, tinnitus, decreased mental functioning, dizziness, visual changes, paresthesias

Question 8

how to treat hyperviscosity?

Answer 8

phlebotomy

Question 9

polycythemia vera clinical characteristics

Answer 9

erythromelalgia 
pruritis 
hypermetabolic state
thrombosis - Budd Chiari syndrome 
Hemorrhage
splenomegaly 
hepatomegaly 
facial plethora

Question 10

Budd Chiari syndrome can develop from what two disorders?

Answer 10

polycythemia vera and PNH

Question 11

phases of polycythemia vera

Answer 11

1. latent- asymptomatic
2. proliferative- hypermetabolic and hyperviscosity
3. spent- anemia, leukopenia, increasing liver and spleen size, fever, weight loss, secondary myelofibrosis
4. AML

Question 12

What mutation is common in patient’s with polycythemia vera?

Answer 12

JAK2

Question 13

Lab findings for polycythemia

Answer 13

• increased hemoglobin hematocrit
• increased platelets
• increased EPO
• positive for JAK2 mutation
• BASOPHILIA - increased basophils present
• increased uric acid –> can lead to gout

Question 14

how does polycythemia usually arise?

Answer 14

from a single neoplastic stem cell, mutations in JAK2

Question 15

treatment for polycythemia?

Answer 15

• phlebotomy
• low dose aspirin
• hydroxyurea

Question 16

90% of patients with essential thrombocytosis have

Answer 16

somatic genetic mutation

ex. JAK2, CALR, MPL

Question 17

essential thrombocytosis similar symptoms to p vera except there is increased risk of

Answer 17

arterial or venous thrombosis

Question 18

essential thrombocytosis clinical symptoms

Answer 18

• pruritis
• erythromelagia
• thrombosis
• hemorrhage
  differences compared to p vera
  1. digital ischemia
  2. no facial plethora

Question 19

first step in diagnosing essential thrombocytosis

Answer 19

• rule out other causes of thrombocytosis
• order a ferritin level
  1. infection
  2. cancer
  3. iron deficiency
  4. bleeding
  5. inflammation

Question 20

who do we treat for essential thrombocytosis?

Answer 20

• patients who are greater than 65
• have greater than 450 platelets on two occasions 1 month apart
• increased risk of thrombosis

Question 21

treatment is focused on

Answer 21

• decreasing platelet count

- hydroxyurea

Question 22

laboratory findings for essential thrombocytosis

Answer 22

• JAK2 mutation
• abnormal irregular platelet, usually giant sized
• increased megokaryocytes
• ferritin level should be normal

Question 23

between the three myeloproliferative disorders which one has the most risk to turn into AML?

Answer 23

myelofibrosis 
then 
polycythemia vera
then 
essential thrombocytosis

Question 24

myelofibrosis is mainly a disorder that

Answer 24

arises from a disorder that affects the clonal stem line for megakaryocytes

Question 25

treatment for myelofibrosis

Answer 25

- there is not definitive treatment - supportive care - splenomegaly to relieve abdominal pain but increased risk of hemorrhage - if young bone marrow transplant

Question 26

clinical features of myelofibrosis

Answer 26

- splenomegaly and heptamegoly - fever, chills, weight loss - extramedullary hematopoesis that causes splenomegaly which can lead to early satiation

Question 27

laboratory findings of myelofibrosis

Answer 27

- blood smear- teardrops, NRBC, and granulocytes, leukoerythroblastic (immature leukocytes) - dry bone marrow tap - early on the RBC, WBC and Hb are all elevated but as the disease progresses they will all decrease

Question 28

all myeloproliferative disorders can result in spent phase which is

Answer 28

similar to myelofibosis

Question 29

myeloid metaplasia

Answer 29

refers to the early proliferative phase of myelofibrosis where there is extrameduallary hematopoiesis

Question 30

myelodysplastic syndrome

Answer 30

- abnormal clonal line that results in cytopenia

Question 31

myelodysplastic syndrome is usually found in the

Answer 31

elderly population due to lower levels on CBC

Question 32

peripheral smear for myeldysplastic syndrome should show

Answer 32

1. bilobed neutrophils 2. monocytosis- increased number of monocytes 3. macrocytosis - with normal levels of B12 and folate

Question 33

bone marrow biopsy should show

Answer 33

1. ringed sideroblast 2. megaloblastic erythropoiesis 3. blast cells should account for less than 20 % of slide (more than 20 would be leukemia) 4. dyserythropoiesis- abnormal nucleus on RBC 5. small megokaryocytes with abnormally hypolobated nuclei

Question 34

mylodysplastic is usually are result of

Answer 34

cytogenetic abnormalities

Question 35

mylodysplastic treatment

Answer 35

- treatment is usually supportive - platelet and rbc transfusion - hypermethylation of DNA - growth factors

Question 36

once the patient develops AML

Answer 36

the chances of achieving remission is much shorter