Myelodisplasia syndrome

• myelodisplasia syndromes are a

group of clonal stem cell disorders
characterized by ineffective
hematopoiesis and an increased risk
of transformation to AML.
• Some or all of the bone marrow is replaced by
the clonal progeny of a multipotent stem cells
that mutant but still differentiate into red blood
cells, granulocytes, and platelets.
• It usually appears hypercellular bone marrow
or blood edges normoseluler but showed
pancytopenia.
MDS arise with the state:
1. Idiopathic or primary MDS
Occurs in patients over the age of 50 years and
this syndrome develop slowly
2. Mode is associated with therapy
Complications of therapy with drugs that are
mielosupresif or radiotherapy and this
syndrome usually appear within 2 to 8 years
after treatment
 Pathogenesis
• Most of them are unknown, but MDS is
typically arise with damage to stem cells.
Primary MDS or therapy-related MDS has a
correlation with the same clonal chromosomal
abnormalities, including monosomy 5 and
monosomy 7, deletion 5q and 7q, trisomy 8
and deletion 20q
 Morphology
The most typical test results are abnormal
differentiation (dysplasia) which is about three blood
cell lineages (erythroid, myeloid, and
megakaryocyte).
o Erythroid
Ringed sideroblasts, eritroblas with full
mitochondrial iron and visible as perinuclear
granules in Prussian blue staining. Maturation
megaloblastoid that seemingly resemble image on
vitamin B12 or folate deficiency.
o Granulocytic
Neutrophil cells with a reduced number of secondary
granules, toxic granulation. Cells pseudo-Pleger-
Huet (neutrophil cells with two nuclei lobes only).
Myeloblasts may increase but less than 20% overall
bone marrow cellularity
o Megakariositik
Lobe nucleus of megakaryocytes with single or
multiple separate nuclei
o Peripheral blood: peripheral blood cells often
contain pseudo-Pelger-Huet, giant platelets,
makrosit, poikilosit and relative or absolute
monocytosis, usually myeloblasts forming less
than 10% of peripheral leukocytes
 Clinical course
Primary MDS typically about those people
over the age of 60 years. Usually not too cause
symptoms, the disease can be determined by a
blood test. History of the disease is dominated
by symptoms stemming from cytopenia,
particularly thrombocytopenia. Progression to
AML occurs in 10% to 40% of cases with
additional clonal cytogenetic changes
overview
 Prognosis
The average life of patients varied from 9 to 29 months
although some patients can live for 5 years or more.
Factors that marks the end of a poor outcome include:
⁻ The development of tumors after cytotoxic therapy.
Therapy-related MDS patients have a more severe
cytopenia and often progresses rapidly to AML
⁻ Increasing the number of blasts in the bone marrow or
blood
⁻ Multiple clonal chromosomal abnormalities
⁻ Severe thrombocytopenia
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