Myelodysplastic Syndromes MDS

Question 1

Therapies related MDS

Answer 1

( radiotherapy , some chemotherapies)

Question 2

Secondary MDS due to

Answer 2

combination of radiation ☢️ +alkylating agents
DNA topoisomerase inhibitors

Question 3

…………. And ………….And ……………. Can evolve into MDS

Answer 3

Aplastic anemia
Fanconi anemia
PNH

Question 4

Cytogenetic abnormalities

Answer 4

11q23 following topoisomerase II inhibitors

Question 5

Trisomy 8 MDS.

Sideroblastic anemia

Answer 5

responds to immunosuppressive therapy
(Eight/Hate / مثبط مناعة)

mutations in mitochondrial genes» ineffective erythropoiesis and disordered iron metabolism

Question 6

Clinical Overlap / Associations

Answer 6

AML
Aplastic anemia
Myeloproliferative disease
LGL leukemia
Autoimmune diseases

Question 7

Acute neutrophilic dermatitis

Answer 7

Sweet ‘s syndrome

Question 8

…………..may become large and lack granules

Answer 8

Platelets

Question 9

……………. contain Döhle bodies; and may be functionally deficient

Answer 9

Neutrophils

Question 10

The total (WBC) count is usually normal or low, except in ……………..

Answer 10

CMML

الوحيدات ليلا فرة زياي بوود

Question 11

Circulating…………… usually correlate with marrow blast numbers

Answer 11

myeloblasts

Question 12

BM At MDS

Answer 12

Megaloblastic nuclei +ringed sideroblasts in the erythroid lineage;

hypogranulation and hyposegmentation in granulocytic precursors, with an increase in myeloblast

megakaryocytes showing reduced numbers or disorganized nuclei.

Question 13

The prognosis strongly correlates with the proportion of .

Answer 13

Marrow blasts

Question 14

Differential diagnosis

IF the BM shows ringed sideroblasts

Answer 14

vitamin B6 deficiency can be assessed by a therapeutic trial of pyridoxine

Question 15

More difficult are the distinctions between hypocellular MDS and………….. or between refractory anemia with excess blasts and…………..

Answer 15

aplasia (A.A)

early acute leukemia

Question 16

The WHO considers the presence of …………..in the marrow as the criterion that separates acute myeloid leukemia (AML) from MDS.

Answer 16

20 % blasts

Question 17

Most patients die as a result of complications of…………..and not due to leukemic transformation; perhaps …………. succumb to other diseases unrelated to their MDS.

Answer 17

pancytopenia
One third

The outlook in therapy-related MDS, regardless of type, is extremely poor, and most patients progress within a few months to refractory AML.

Question 18

The median survival varies greatly from years for patients with…………… to a few months in…………..

Answer 18

5q- or sideroblastic anemia

refractory anemia with excess blasts or severe pancytopenia associated with monosomy 7

Question 19

TREATMENT OF MDS
Only stem cell transplantation offers cure.

Answer 19

but older patients are particularly prone to develop treatment related mortality and morbidity.
For older patient with MDS excess blast  DNA Hypomethelating agent (HMA)Azacitidine OR Decitabine (more potent).

For MDS 5q-  Lenalidomide

For low risk MDS (without blast) and HLA-DR15 +ve  immunosuppression( ATG , cyclosporine ).

ATG+the anti-CD52 monoclonal antibody (Alemtuzumab) is especially effective in (younger than age 60 years) and who bear the HLA-DR15.

RBC transfusion support should be accompanied by iron chelation to prevent secondary hemochromatosis