22) MDS Flashcards
primary characteristics of MDS
- PB cytopenia
- cellular maturation abnormalities (dyspoiesis)
- BM hyperplasia
excessive apoptosis in the BM
MDS
MDS incidence
most common >50 years
60% males
3-13 per 100,000
most common finding in MDS
anemia
the only cell that might be ↑ in MDS is…
monos
MDS usually leads to a ———– anemia
macrocytic (similar to megaloblastic)
may be dimorphic pop
will NOT be present in granulocytes with MDS
- toxic gran
- dohle bodies
- vacuolization
signs of dysgranulopoiesis
- agranular/hypogranular
- bluer cytoplasm
- pseudo-PH
- hypersegmentation
- donut shape nucleus
- bright pink granules
- dense blue rim in cytoplasm
- Auer rods
- ↓ enzyme activity
- functional defects
signs of dysthrombopoiesis
- giant plts
- hypogranular plts
- large granules
- micromegakaryocytes
- abnormal megakaryocyte nucleus shape
in MDS, …… in BM ↑ risk of leukemia
central clusters of myeloid precursors
———- hyperplasia most common in MDS
erythroid
signs of dyserythropoiesis
- megaloblastoid nucleus/asynchrony
- giant, multinucleated erythroblasts
- nuclear budding
- karryhorhexis
- vacuoles or stippling
- ringed sideroblasts
iron, B12, folate in MDS
- serum iron normal or ↑
- TIBC normal or ↓
- B12/folate normal or ↑
dysgranulopoiesis may lead to blasts with…
few granules
MDS-SLD
MDS with single lineage dysplasia
cytopenia in 1+ cell lines, dysplasia in only 1 cell line
MDS-SLD
most common MDS-SLS
and others
refractory anemia
refractory neutropenia
refractory thrombocytopenia
refractory anemia from MDS-SLD is usually…
normocytic or macrocytic
MDS-RS
MDS with ringed sideroblasts
2 types of MDS-RS
- MDS-RS-SLD
- MDS-RS-MLD
SF3B1
MDS-RS
requirements for MDS-RS
5-15% RS and SF3B1 mutation
OR
>15% RS
MDS-MLD
MDS with multilineage dysplasia
dysplasia in at least 10% of cells in 2+ lineages
MDS-MLD
30-40% of all MDS cases
MDS-MLD
MDS-MLD may progress to…
AML
MDS-EB
MDS with excess blasts
blast count >5% in BM, cytopenia in 2+ lineages, dysplasia in all 3 lineages
MDS-EB
most aggressive MDS and likely to progress to acute leukemia
MDS-EB
2 types of MDS-EB
- MDS-EB1: 5-9% blasts in BM, no auer rods
- MDS-EB2: 10-19% blasts in BM, auer rods
MDS with good prognosis and low risk of progression
70% cases are women
MDS with isolated del(5q)
deletion of long arm of chromosme 5
MDS with isolated del(5q)
sign of MDS with isolated del(5q) in PB/BM
hypolobulated megakaryocytes
3 types of MDS-U
- with 1-4% PB blasts
- with single lineage dysplasia
- based on defining cytogenetic abnormality
MDS of childhood involving persistent cytopenias and dysplasia in any or all lines
refractory cytopenia of childhood
proliferation + dysplasia
MDS/MPN
monocytosis, far left shift, and absence of BCR/ABL1
CMML
↑ lysozyme (from monos)
CMML
> 20% monos in BM
CMML
types of CMML
- CMML1: <10% blasts in BM
- CMML2: 10-19% blasts in BM
CML with no detectable BCR/ABL1 and dysplasia
aCML
aCML
atypical CML
CMML in children
no BCR/ABL1
JMML
JMML
juvenile myelomonocytic leukemia
75% dx in children <3yo
can be in kids 1 month to adolescent
JMML
MDS/MPN-RS-T
MDS/MPN with ringed sideroblasts and thrombocytosis
↑↑ plt
>15% ringed sideroblasts
JAK2 mutation
MDS/MPN-RS-T
only cure for MDS
BM transplant
