41: Myeloproliferative Disorders - Hilgerson

Question 1

4 types of myeloproliferative disorders

Answer 1

• CML- chronic myelogenous leukemia
• polycythemia vera PCV
• chronic idiopathic myelofibrosis
• essential thrombocythemia

all can eventually progress to AML

Question 2

__________ tends to occur in all of the myeloproliferative syndromes

Answer 2

basophilia

there shouldn’t be many basophils normally

Question 3

only known etiology for CML

Answer 3

ionizing radiation

Question 4

blood count 65000 WBC might be… (leukocytosis)

Answer 4

chronic phase CML

can potentially go on to accelerated and blast phases

Question 5

philadelphia chromosome is associated with..

Answer 5

CML

philadelphia chromosome (bcr-abl oncogene 9,22)

Question 6

clinical presentation of CML

Answer 6

hepato-splenomegaly, low alk phos, weight loss, increasing abdomen growth…. if they are symptomatic

Question 7

of all CML pts _______ per year get ALL/AML

Answer 7

25%

more likely AML

Question 8

Hb and Hct above normal =

Answer 8

polycythemia vera

Question 9

relative polycythemia =

Answer 9

caused by dehydration from GI loss or insensible fluid loss

Question 10

low EPO =

Answer 10

PCV

primary erythropoietin independent red blood cell production (EPO independence)

secondary would be exogenous EPO or excessive EPO production

Question 11

intense itching after bathing “pruritic”

Answer 11

PCV

ruddy complexion after bathing, splenomegaly

check their CBC and Hgn/Hct are elevated with elevated RBC mass

Question 12

treatment PCV

Answer 12

periodic phlebotomy with goal Hct less than 45%

Question 13

major cause of death PCV

Answer 13

thrombosis

Question 14

the classic patient with PCV may present with sx of a HA, elevated Hgb/Hct elevated EPO and an elevated RBC mass

Answer 14

false. decreased EPO ****

Question 15

“tear drop” RBC

Answer 15

chronic idiopathic myelofibrosis

Question 16

“dry tap” bone marrow

Answer 16

chronic idiopathic myelofibrosis

characteristic dry tap due to increased collagen and reticulin fibers

Question 17

occurs when neoplastic stem cells migrate and make marrow in areas outside of bone marrow

Answer 17

chronic idiopathic myelofibrosis aka MMM

Question 18

platelets 600 k to 4 million (normal up to 300,000)

Answer 18

essential thrombocythemia

a myeloproliferative disorder with persistent thrombocytosis that is not reactive to another disorder and is not caused by another myeloproliferative disorder

Question 19

bone marrow shows… with essential thrombocytemia

Answer 19

megakaryocyte hyperplasia and clustering

Question 20

complications of essential thrombocythemia

Answer 20

thrombosis (most often)
hemorrhage
splenomegaly

Question 21

*****25% conversion risk

Answer 21

CML

Question 22

***least common and aggressive

Answer 22

essential thrombocytopenia

Question 23

**myeloproliferative disorders see elevated

Answer 23

basophils