myelodysplastic syndromes

Question 1

MDS:
a. it is a __________ disease

b. characterized by:
1. cytopenia with a hypercellular ________
2. dysplasia
3. __________in one or more of the major myeloid cell lines

c. associated with a variable progression to ____________

Answer 1

a. it s a clonal hematopoietic stem cell disease

b. characterized by:
1. cytopenia witha hypercellular Bone marrow
2. dysplasia
3. ineffective hematopoiesis in one or more of the major myeloid cell lines

c. associated with a variable progression to acute myeloid leukemia

Question 2

Bilogical features of MDS:

1. Stem cells exhibit a
   ________ capacity for self renewal and differentiation
2. There is abnormally ________ cell death in MDS marrow that may partially explain the ineffective hematopoiesis

Answer 2

1. Stem cells exhibit a defective capacity for self renewal and differentiation
2. There is abnormally increased cell death in MDS marrow that may partially explain the ineffective hematopoiesis

Question 3

approximately 90% of MDS cases are de novo due to no identifiable cause but potential risk factors include exposure so we need to ask about occupational history

Answer 3

yep

Question 4

MDS clinical presentation:

1. commonly in ____ patients
2. initial finding of ________, bleeding, easy bruising and fatigue
3. many patients appear first as ______ and it is established through lab reports looking at; (3)

Answer 4

1. commonly in older patients
2. initial finding of anemia, bleeding, easy bruising and fatigue
3. many patients appear first as asymptomatic and it is established through lab reports looking at; (1) neutropenia, (2) anemia and (3) thrombocytopenia

Question 5

Blood smear in MDS

Answer 5

1. macrocytosis and aniocytosis of RBC

2. Pseudo Pleger-Huet cells - dysplastic neutrophil with a bilobed nucleus joined by a bridge

Question 6

In MDS, the bone marrow is usually _________ at diagnosis and early abnormal myeloid progenitors are identified in the marrow in varying percentages and small ___________ may be seen in the marrow as well.

In this disease there is an increase in what cell in the bone marrow? and what is the percentage and why is the percent important?

Answer 6

In MDS, the bone marrow is usually hypercellular at diagnosis and early abnormal myeloid progenitors are identified in the marrow in varying percentages and small megakaryocytes may be seen in the marrow as well.

In this disease there is an increase in what cell in the bone marrow- myeloblasts are increased but less than 20% b/c a count over 20% means that the patient is more likely to have ALM

Question 7

50% of patients with MDS have a detectable cytogenetic abnormality

Answer 7

yep , most common deletion are 5. or 7 or there is a trisomy 8

Question 8

MDS classification is based on:

1. percentage of ________in the BM
2. dysplasia: ____________involved and degree
3. presence/absence of ________
4. presence and type of _________ abnormality

Answer 8

1. percentage of blasts in the BM
2. dysplasia: lineages involved and degrees
3. presence/absence of ringed sideroblasts
4. presence and type of cytogenetic abnormality

Question 9

at least 2 cytopenias with BM dysplasia in those lineages

Answer 9

RCMD

Question 10

isolated anemia, erythroid dysplasia, w. ringed siderblasts

Answer 10

RARS

Question 11

at least one cytopenia/ dysplasia, 5-19% blasts

karyorrhexis- degeneration of the progenitor

Answer 11

RAEB-2

Question 12

BM blast percentage is important in classifying MDS

Answer 12

yep

Question 13

cytogenetics in MDS:

1. clonal chromosomal abnormalities detected in bone marrow cells in _______% of pts with primary MDS
2. most common aberrations are del___, monosomy ___ or del(____)
3. abnormalities of 11q23 are common in T-MDS associated with _________therapy

Answer 13

1. clonal chromosomal abnormalities detected in bone marrow cells in 40-70% of pts with primary MDS
2. most common aberrations are del(5q), monosomy 7 or del(7q)
3. abnormalities of 11q23 are common in T-MDS associated with topo inhibitor therapy

Question 14

prognosis for MDS is directly related to

1. number of BM _______ cells
2. certain _________ abnormalities
3. the amount of peripheral blood _____

Answer 14

1. number of BM blast cells
2. certain cytogenetic abnormalities
3. the amount of peripheral blood cytopenias

Question 15

MDS are reclassified as acute myeloid leukemia with myelodysplastic features when blood or BM reach or exceed 20%

Answer 15

yep

Question 16

when MDS transition to AML there is a higher chance of death but the chemo is more responsive

Answer 16

false

many patients succumb to complications of cytopenias before progression to AML

the acute leukemic phase is less responsive to chemo than is de novo AML

Question 17

1. blasts in both blood and BM are less than 5%
2. subtype is associated with a long survival
3. overrepresentation of females
4. megakaryocytes with hypolobated nuclei
5. refractory macrocytic anemia
6. nl or increased platelet count

Answer 17

MDS associated with an isolated del(5q) chromosome abnormality

Question 18

treatments for MDS:

1. supportive care includes transfusion with __________therapy and growth factors
2. _____________ such as lenalidomide for 5q-MDS
3. __________such as decitabine
4. _________ induction type therapy
5. ___________ transplantation

Answer 18

1. supportive care includes transfusion with iron chelating therapy and growth factors
2. disease modifying agents such as lenalidomide for 5q-MDS
3. DNA methyltransferase inhibitors such as decitabine
4. AML induction type therapy
5. allogeneic hematopoietic stem cell transplantation

Question 19

what is the big deal of lenalidomide for treatment of 5q-syndrome

Answer 19

lenalidomide induced transfusion independence and there is the potential for complete cytogenetic remission