MYELODYSPLASTIC NEOPLASM Flashcards
(45 cards)
1982 :______ proposed terminology & a specific set of morphologic criteria of MDS
FAB
______
1997: includes molecular, cytogenetic, and immunologic criteria in addition to morphologic features
2022: renamed MDS as_____
WHO
myelodysplastic neoplasms
• are group of acquired clonal hematologic disorders
MDS
MDS
(peripheral blood FINDINGS??
progressive cytopenias
MDS defects in THE MATURATION OF?
erythroid, myeloid, and/or megakaryocytic maturation
MDS
•_____: median age at diagnosis
• rarely in age____
77
40
ETIOLOGY
• Phenotypically normal_____ is the cell of origin for MDS
• Mutations in the affected HSCs drive clonal expansion at the expense of normal HSCs
HSCs
MDS
• These mutations are usually______ (acquired during life, not inherited) and accumulate with_____, leading to an increased risk of MDS.
somatic
aging
• In MDS, these cells acquire mutations that lead to______, meaning the mutated cells outcompete normal HSCs.
clonal expansion
- clonal disorders (do not meet the criteria for classification as MDS)…
Clonal Cytopenia of Unknown Significance (CCUS)
Clonal Hematopoiesis of Indeterminate Potential (CHIP)
HSC mutations that lead to MDS
(4)
- primary MDS
- myeloid neoplasm post cytotoxic therapy
- secondary to exposure to chemicals or radiation (no prior disease treatment)
- Inherited - myeloid neoplasms
• Most common form
• Develops without prior exposure to chemotherapy, radiation, or inherited mutations.
Primary (De Novo) MDS
• Occurs after exposure to DNA-damaging treatments like chemotherapy or radiation therapy.
• Median onset: 4 to 7 years after treatment.
• Has a poor prognosis and can progress rapidly to AML.
Therapy-Related MDS (Myeloid Neoplasm Post-Cytotoxic Therapy - MN-pCT)
• Can be caused by exposure to chemicals or radiation without prior treatment for another disease.
• Examples: Benzene, pesticides, radiation accidents.
Secondary MDS (Environmental Exposure)
• Some people inherit genetic mutations that make them more susceptible to developing MDS.
Inherited MDS (Germline Mutations)
• Occurs after exposure to DNA-damaging treatments like chemotherapy or radiation therapy.
• Median onset: 4 to 7 years after treatment.
• Has a poor prognosis and can progress rapidly to AML.
• Patients treated with growth factors (e.g., G-CSF, GM-CSF) for bone marrow stimulation are also at increased risk.
Therapy-Related MDS (Myeloid Neoplasm Post-Cytotoxic Therapy - MN-pCT)
• Can be caused by exposure to chemicals or radiation without prior treatment for another disease.
• Examples: Benzene, pesticides, radiation accidents.
Secondary MDS (Environmental Exposure):
Inherited MDS (Germline Mutations):
• Some people inherit genetic mutations that make them more susceptible to developing MDS.
• Common in conditions like:
• ______
• Fanconi anemia
• Diamond-Blackfan anemia
• Shwachman-Diamond syndrome
Down syndrome (higher risk of developing myeloid neoplasms)
MDS is characterized by:
- presence of progressive_____ despite a_____ bone marrow,
- ineffective _____,
- dysplasia in one or more cell lines
- clonal hematopoeisis (presence of cytogenetic abnormalities)
cytopenias; cellular
hematopolesis
DYSERYTHROPOIESIS
- most common morphologic finding
Oval macrocytes
- seen in the presence of normal vitamin B12 and folate
Oval macrocytes
Dyserythropoiesis
- Hypochromic microcytes (in adequate iron stores) - seen in MDS
Oval macrocytes
Dyserythropoiesis
DYSERYTHROPOIESIS
• Oval macrocytes
Dimorphic RBC
Ringed sideroblast
Megaloblastoid
