Myelodysplasias Hubbard Slides

Question 1

clinical picture of myelodystplastic syndromes

Answer 1

pancytopenia with hyperplastic marrow

-potential risk for development of acute leukemia

Question 2

etiology of myelodysplasias

chemo

Answer 2

pts treated for acute leukemia
pts treated for hodgkin and NHL
pts treated for breast cancer

Question 3

offending agents causing myelodysplasias

Answer 3

alkylating agents- cyclophosphamide, ifosfamide, cisplatin, carboplatin, nitrogen mustard

antracyline antibiotics (rubicins)

Question 4

etiology of MDS

Answer 4

chemo
radiation
cytogenetics

Question 5

cytogenetics of MDS

Answer 5

partial or total loss of long arm of chromosome 5 or 7
inversion of chrom 16
trisomy 8

Question 6

age of pts with myelodysplasias

Answer 6

not usually younger than 55

Question 7

lab values Myelodysplasias

LDH
ferritin
serum fe and TIBC

Answer 7

LDH increased
ferritin increased
FE and TIBC normal

Question 8

in patients with pancytopenia if you suggest what 3 things you my friend, will look like a rockstar

Answer 8

hypersplenism
aplastic anemia
myelodysplasia

Question 9

refractory anemia with ringed sideroblasts is what

• decreased what
• can be caused by what
• check this how

Answer 9

anemia that is refractory to typical medical therapy

• decreased reticulocyte count
• can be from pyridoxine deficiency (vit B6) or MDS
• check B6 levels
• replace B6 for at least 6 months if deficient, then repeat marrow, if no improvement has pyridoxine resistant sideroblastic anemia

Question 10

adverse prognostic features of myelodysplasias

Answer 10

marrow blasts > 5%
platelets under 100 k
hemoglobin under 10
neutrophils under 2500
age over 60

Question 11

poor prognosis cytogenetically of MDS

Answer 11

monosomy 7

hypodiploidy

Question 12

good prognosis MDS

• response to what
• give what with this drug

Answer 12

5q syndrome
responds to lenalidomide
-give aspirin, warfarin, plavix, to prevent thrombosis with lenalidomide

Question 13

supportive care of MDS

Answer 13

avoid meds that damage marrow
aggressive treatment of infections bc of neutropenia
transfuse PRBCs when symptomatic
transfuse platelets only for bleeding or in surgery prep
B6 if needed
EPO only if serum level of EPO is below 500

Question 14

what supprtive care for MDS is reasonable to try if no other options exist

Answer 14

androgens

Question 15

low/intermediate intensity therapy for MDS

Answer 15

hypomethylating agents

• decitabine and azacitidine
• lenalidomide in 5q syndrome only

Question 16

high intensity therapy for MDS

Answer 16

AML induction style treatment

HSC transplant for pts under 60 and have sibling matching donor

Question 17

triad for myelofibrosis

Answer 17

leukoerythroblastic anemia
poikilocytosis (abnormal shaped RBCs)
splenomegaly

Question 18

what do you see increased in marrow in MF

Answer 18

reticulin deposition (PDGF)
increased marrow MKC

Question 19

what indicates development of later stages of disease for MF

Answer 19

pancytopenia and organomegaly

Question 20

treatment for MF in young pt doing well

Answer 20

BM transplant

Question 21

treatment for pancytopenia in MF

Answer 21

manage anemia with transfusions
EPO in levels below 500
transfuse platelts with bleeding occuring
GFs for neutropneia
treat infections aggressively

Question 22

most common cause of death in MF

Answer 22

overwhelming infection

Question 23

treating splenomegaly in MF

Answer 23

hydroxyurea
radiation
in severe cases, splenectomy

Question 24

jak inhibitor treatment for MF

Answer 24

Ruxolitinib***