Myelodysplastic disorders

Question 1

classification of myelodysplastic syndrome:

Answer 1

refractory cytopenia with single lineage dysplasia
refractory anemia with ringed sideroblasts
refractory cytopenia with multilineage dysplasia
refractory anemia with excess blasts
chronic myelomonocytic leukemia
refractory anemia with excess blasts in transformation

They have to be low.

Question 2

Signs and symptoms of myelodysplastic syndrome

Answer 2

can be asymptomatic
anemia symptoms (fatigue, weakness, angina)
thrombocytopenia findings (easy bruising) 
granulocyte dysfunction (recurrent infections)

Question 3

laboratory findings of myelodysplasia

Answer 3

normocytic or macrocytic anemia with low reticulocytosis, leukopenia, thrombocytopenia, possible blasts
hypercellular bone marrow w/ single or multilineage dysplasia

Question 4

How do we know if a patient with anemia has MDS?

Answer 4

get a peripheral smear
rule out other causes
Needs bone marrow biopsy

If there’s anemia with elevated MCV and normal vitamin B12, folate, TSH levels and not on any medication that can cause high MCV (hydroxyuria or zidovudine) consider MDS given his age

Question 5

What is myelodysplastic syndrome?

Answer 5

bone marrow doesn’t work well so can’t make blood cell lines very well and has hypercellarity and can transform into AML.

group of malignant hematopoietic stem cell disorders with progressive bone marrow failure leading to blood cell dysplasias and cytopenias.

Question 6

How are most MDS cases found?

Answer 6

some are seen on routine lab work and other people present with fatigue, infection, or easy bruising or bleeding.

Question 7

if anemia is presenting feature of MDS, what do you see on labs?

Answer 7

macrocytic or normocytic anemia w/ inappropriately low reticulocyte count,

leukopenia about 5-% with monocytosis,

thrombocytopenia (25%) and thrombocytosis (8% of time)

Question 8

how do we diagnose formally MDS?

Answer 8

diagnosis of exclusion;

rule out other causes of cytopenia (medications, vitamin B12/folate/copper deficiency,)

MUST get a bone marrow biopsy for diagnosis

bone marrow biopsy will give prognosis of MDS.

Question 9

What do you see on bone marrow biopsy with MDS?

Answer 9

hypercellular marrow with single or multilineage dysplasia (peripheral blood smear cytopenias happen with increased intramedullary apoptosis)

Question 10

How to classify and risk stratify and stage dx of myelodysplasia?

Answer 10

cytogenetics.

Question 11

ETOH effect on MCV?

Answer 11

causes elevated macrocytosis but rarely doesn’t have MCV>110

Question 12

Anemia and MCV>110, can vitamin B12 deficiency play a role

Answer 12

If vitamin B12 level is >300 pg/ml it’s unlikely deficient; would see hyperlobulated neutrophils on peripheral smear.

Question 13

What can cause iatrogenic MDS and myeloproliferative neoplasms and AML?

Answer 13

doxorubicin or anthracycline induced leukemia

presents 5 to 10 years after treatment and they start with a preceding MDS phase.

Question 14

what is a myelodysplastic syndrome

Answer 14

clonal stem cell disorders with INEFFECTIVE hematopoiesis leading to dysplastic hypercellular bone marrow and peripheral blood cytopenias

can’t make proper blood cell lines and so get hyperfunctioning bone marrow making crappy junk.

Question 15

in order to rule that there is myelodysplastic syndrome what needs to be ruled out?

Answer 15

vitamin B12, folate, copper deficiency
ETOH consumption

infections like HIV and medication use.

Question 16

most common cytopenia with myelodysplastic syndrome?

Answer 16

macrocytic anemia

blood cells don’t function well and so at greater risk for bleeding and infection.

some can be transfusion dependent if symptomatic.

Question 17

peripheral smear of myelodysplastic disorder:

Answer 17

see hypogranular neutrophils and platelets (bone marrow not functioning well)

need to also check peripheral blood smear (ovalomacrocytosis, hyposegmented neutrophils)

Question 18

prognosis of MDS is based on

Answer 18

bone marrow biopsy

Ex1. few blast cells on bone marrow biopsy and reasonable peripheral blood cell counts - median survival is 9 years

Ex2. >10% blasts on bone marrow biopsy and severe pancytopenia (Hgb<10, Abs neutrophil count: <800 and plt<50K median survival <1 yr

Question 19

only curative treatment for MDS is

Answer 19

allogenic hematopoietic stem cell transplant

most pts don’t qualify because they are too old.

Question 20

goals of treatment for myelodysplastic syndrome are:

Answer 20

treatment of symptomatic cytopenias

reduce risk for progression to AML

symptomatic anemia - transfusions and EPO agents

Question 21

What is the percentage of the people who have MDS and develop AML in 1st year ?

Answer 21

25%

Question 22

how to treat pts who have MDS that converts to AML?

Answer 22

treat with azacytidine and decitabine - decreases dependence on RBC transfusions.

MDS with 5q cytogenic abnormality can be treated with lenalidomide if transfusion dependent

Question 23

When do we use lenalidomide for treatment?

Answer 23

for MM and MDS with 5q - syndrome.

Lenalidomide is a immunodulator and anti-angiogenic agent. increases risk for VTE.

Question 24

what do you see on peripheral smear with myelodysplastic syndrome?

Answer 24

anisocytosis, Pelger Huet anomaly and see hyposegmented neutrophils.