10. Myelodysplastic syndromes: pathogenesis, genetics, clinical manifestation, diagnosis, treatment.

Question 1

how is AML and MDS diffentaited ?

Answer 1

FAB seperates AML and MDS according to the perectage of blasts in the bone marrrow

Question 2

myelodysplastic syndrome can turn int which type of leukmeia commonly

Answer 2

MDS is an intermediate stage for for acute myelid leukemia

IT CAN TURN IO ACUTE MYELOID LEUKEMIA less than 20 percent of blast

Question 3

myleoproliefratve and myelodisplastic sydrome usually occurs in wha age

Answer 3

elderdly of 70 years old

Question 4

what isthe clinical manifestation

Answer 4

fatgue - anemai

bleeding = thrombocytopena

granulocytopnea - infections

Question 5

what is the diagnosis of MDS ?

Answer 5

pancytopenea
with
MCV is high due to erythroblast

hypercelular marrow with pancytopnea

peripheral smear

erythroid lineage :
basophilic stiplling of the rbc = associated with = sideroblastic anemia
dimporhic red blood cells - macrocytic and microcytic = rdw is high

myeloid
pseudo pelger heut = only bilobed granulocyte
auer rods
hypogranulated

megakaryocyte 
large agranuluar platelets 
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staining the same as AML 
romaowsy 
auer rods fund on mpo 

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bone marrow biopsy 
blasts less than 20 
hypercellular marrow 
dysplastic of atleast 10 percent  of specific myleoid lineage 
and cytopnea   

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fish analysis detects traslocation and deletion

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flow cytometry = myeloid blast express = cd34 ,33 ,13

Question 6

what is the cause of MDS

Answer 6

de novo

or acquired - chemotherapy
radiotherapy
benzene

Question 7

what isthe classification of MDS ?

Answer 7

refactory anemia = slowest subtype to trasnform to aml

the amount of blast in marrow is less than 5 percent

and peripheral blast less than or equal to 1

morphological abnormalities confined to erythroid lineage

and requires 6 months of anemia

or morphological abnormalities present present n two or more lineage but still blast is less than 5 percent
refactory cytopena with multilineage dysplasia

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refactory anemia with ringed sideroblasts
marrow blasts is less than 5 percent
peripheral blast is less than or equal to 1percnt
and ringed sideroblast is more than 15 percent

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chronic myelomonocytic

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5q syndrome 
severe macrocytic anemia 
variable neutropnea 
elevated or normal platleets with hpolobulated megakaryocytes 

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refactory anemia with excess blasts
marrow blast is 5-20 percent
peripheral blast less than 5 percent

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refactory anemia is excess blast in transformation
marrow blast less than 30 percent
peripheral blast more than 5 percent
aeur rods now present

Question 8

what are the unfavouralble prognostic factors for MDS

Answer 8

increased percentage of blasts but less than 20 percent

cytopena increased

increased age

cytogenic abnormailty with chormosome 7

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favoruable 5q deletion in MDS

Question 9

what is the treatment of MDS ?

Answer 9

supportive therapy
if old and highly advanced

for anemia = erythropoetin growth factors = epoetin
blood transfusion = (iron over loas - chelation by deforxamine)

thrombocytopnea - human growth factors = romiplastin , elthrombopag
platlet trasnfusion
aminocaproic acid

leukopena - humarn growth factors
pegfilgrastin = granulocyte

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demethalating agents
azacitidine
AZACITIDINE

standard chemo in high risk MDS
cytarabine

immunomodulating
lenalidomide = for 5 q deletion

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only potential cure
stem cell transplant
destroy bone marrow with chemotherapy and radiation give them stem cells