Myelodysplastic Disorders Flashcards
myelodisplastic syndromes
heterogenous group of clonal hemtaological malignancies characterized by
- PB cytopenias
- Propensity to transform into acute leukemia
- Dysplastic features
Formerly called “preleukemic anemia” or “refractory anemia”
Cytopenia - caused by ineffective hematopoiesis
Comes back to our apoptosis balance
- abnormal decrease in apoptosis leads to cell accumulation
- abnormal increase in apoptosis leads to decresed cellular output
In MDS -> there is an abnormal increase in intrameduallary apoptosis in BM
- due to an imbalance between cytokines
anti-apoptotic
GM-CSF
Apoptotic Increasing
- Fas ligand
- Fas Ag
- TGF-beta
- TNF alpha
PB dyserythropoiesis
- internuclear bridging (BM)
- anisocytosis with macrocytosis
- howell jolly body
- basophilic stippling
- teardrop cell
BM dyserythropoiesis
- asynchronous development
- nuclear budding
- internuclear bridging
- basophilic stippling
dysgranulocytosis PB
- hyposegmentation
- bilobulation (pseudo-Pelger-Huet)
- Hypogranulation
dysgranulocytopoitesis BM
hyposegmentation (pseudo-pelger-huet)
hypogranulation/agranulation
dysmegakaryocytosis PB
giant platelet
dysmegakaryocytopoiesis BM
- megakaryotcytoes with detached nuclei
- hypogranulation
MDS phenotype changes as disease progresses toward leukemia
BM
- blasts increase
PB
- Leukopenia changes to leukocytosis
Hepatosplenomegaly can progress
MDS evolves into a proliferative blood disorder
MDS affects
predominantly the elderly
- median age 70
Men are effected more
can be seen following a viral infection
can occur in patients treated with chemotherapy and radiation
- secondary MDS
MDS genetic abnormnalities
LOSS of genetic matieral
- chromosome deletions involving part of or whole chromosome
This is the opposite of Acute leukemias which are translocations
MDS clinical features
BM failure
- anemia
- neutropenia
- thrombocytopenia
Symptoms
- fatigue
- recurrent infections
- bleeding
- fever
cytopenia definition
Hgb
PLT
Absolute neutrophil count
monocyte
Single Lineage Dysplasia - MDS
Dysplastic lineages
Cytopenias
BM
PB
Genetics
Dysplastic Lineage: 1
Cytopenias: 1-2
BM : <5% Blasts
PB: <1% Blasts
Multilineage Dysplasia
Dysplastic lineages
Cytopenias
BM
PB
Genetics
Dysplastic lineages: 2-3
Cytopenias: 1-3
BM: <5% Blasts
PB: <1% Blasts
Genetics
Ringed Sideroblasts and single lineage Dysplasia
Dysplastic lineages: 1
Cytopenias: 1-2
BM: <5% blasts, more or equal to 15% Ringed Sideroblasts
PB: <1% Blasts
Genetics
Ringed Sideroblasts and multilineage dysplasia
Dysplastic lineages: 2-3
Cytopenias: 1-3
BM: <5% Blasts, more than or equal to 15% ringed sideroblasts
PB: <1% Blasts
Genetics
Ringed sideroblasts
> 10 iron granules covering <1/3 nuclear rim
Excess Blast 1
Dysplastic lineages: 1-3
Cytopenias: 1-3
BM: 5-9% Blasts
PB: 2-4% blasts
Genetics
Excess Blasts 2
Dysplastic lineage: 1-3
Cytopenias: 1-3
BM: 10-19% Blasts or Auer rods
PB: 5-29% Blasts or Auer Rods present
Isolated del(5q)
dysplastic lineages: 1-3
Cytopenias: 1-2
BM: <5% blasts
PB: <1% Blasts
Genetics: Sq deletion
