MDS FLASHCARDS

*Question: What is the median age at diagnosis of Myelodysplastic Neoplasms (MDS)?
A) 55 years old
B) 65 years old
C) 77 years old
D) 85 years old

*Answer: C) 77 years old

*Question: Which of the following BEST defines Myelodysplastic Neoplasms?
A) Inherited disorder causing excessive production of mature blood cells
B) Acquired clonal hematologic disorder with progressive cytopenias reflecting defects in myeloid maturation
C) Viral-induced destruction of peripheral blood cells
D) Autoimmune disorder targeting megakaryocytes only

*Answer: B) Acquired clonal hematologic disorder with progressive cytopenias reflecting defects in myeloid maturation

*Question: What is the ONLY cytogenetic abnormality specific to MDS?
A) t(9;22)
B) del(7q)
C) del(5q)
D) trisomy 8

*Answer: C) del(5q)

*Question: What is the WHO threshold for dysplasia to be considered significant in MDS?
A) At least 5% of cells in a lineage
B) At least 10% of cells in a lineage
C) At least 15% of cells in a lineage
D) At least 20% of cells in a lineage

*Answer: B) At least 10% of cells in a lineage

*Question: What is the ONLY curative treatment for MDS?
A) Lenalidomide
B) Azacitidine
C) Hematopoietic Cell Stem Transplant (HCST)
D) Luspatercept

*Answer: C) Hematopoietic Cell Stem Transplant (HCST)

*Question: Which MDS subtype has the BEST prognosis?
A) MDS-biTP53
B) MDS-IB2
C) MDS-SF3B1
D) MDS with Multilineage Dysplasia

*Answer: C) MDS-SF3B1

*Question: Which MDS subtype has the WORST prognosis?
A) MDS-5q
B) MDS-SF3B1
C) MDS-LB single lineage
D) MDS-biTP53

*Answer: D) MDS-biTP53

*Question: What is the cell of origin in MDS?
A) Phenotypically abnormal HSCs with visible mutations
B) Phenotypically normal HSCs harboring somatic mutations
C) Mature myeloid cells undergoing dedifferentiation
D) Lymphoid progenitor cells

*Answer: B) Phenotypically normal HSCs harboring somatic mutations

*Question: Which pre-MDS condition is characterized by clonal mutation with LACK of dysplasia and peripheral cytopenia?
A) CCUS
B) CHIP
C) MDS-LB
D) MDS-h

*Answer: B) CHIP

*Question: Which pre-MDS condition lacks dysplasia and an MDS-related mutation?
A) CHIP
B) MDS-h
C) CCUS
D) MDS-IB1

*Answer: C) CCUS

*Question: How many years after cytotoxic therapy does therapy-related MDS typically develop?
A) 1–2 years
B) 2–3 years
C) 4–7 years
D) 8–10 years

*Answer: C) 4–7 years

*Question: What percentage of de novo MDS cases have chromosomal abnormalities?
A) 20%
B) 35%
C) 50%
D) 70%

*Answer: C) 50%

*Question: What is the classic PBS finding of dyserythropoiesis in MDS?
A) Hypochromic microcytes with low ferritin
B) Oval macrocytes with LOW B12 and folic acid
C) Oval macrocytes with NORMAL B12 and folic acid
D) Target cells with elevated bilirubin

*Answer: C) Oval macrocytes with NORMAL B12 and folic acid

*Question: A ring sideroblast is defined as an erythroid precursor with iron granules encircling at least what fraction of the nucleus?
A) 1/4
B) 1/3
C) 1/2
D) 2/3

*Answer: B) 1/3

*Question: How many iron granules minimum must be present per cell for it to be classified as a ring sideroblast?
A) 3
B) 4
C) 5
D) 7

*Answer: C) 5

*Question: Which gene mutation is associated with ring sideroblasts and the most favorable prognosis in MDS?
A) TP53
B) TET2
C) SF3B1
D) ASXL1

*Answer: C) SF3B1

*Question: What inflammatory cell death pathway is responsible for cytopenias in MDS despite a cellular bone marrow?
A) Apoptosis
B) Necrosis
C) Pyroptosis
D) Autophagy

*Answer: C) Pyroptosis

*Question: Overexpression of which receptors on HSCs in MDS leads to myeloid bias and mutation accumulation?
A) CD34
B) TLRs (Toll-like receptors)
C) BCR
D) EPO receptors

*Answer: B) TLRs (Toll-like receptors)

*Question: Which of the following is a classic exam trap regarding MDS morphology?
A) Ring sideroblasts indicate iron deficiency
B) Hypochromic microcytes always indicate iron deficiency
C) Agranular myeloid cells in BM are blasts
D) Oval macrocytes with normal B12/Folate suggest MDS

*Answer: D) Oval macrocytes with normal B12/Folate suggest MDS

*Question: In dysmyelopoiesis, absence of primary granules in myeloid cells can be mistaken for which cell type?
A) Lymphoblasts
B) Blasts
C) Monocytes
D) Plasma cells

*Answer: B) Blasts

*Question: Which of the following is a morphologic feature of dysmyelopoiesis seen on PBS?
A) Oval macrocytes
B) Ring sideroblasts
C) Pseudo-Pelger Hüet anomaly
D) Micromegakaryocytes

*Answer: C) Pseudo-Pelger Hüet anomaly

*Question: Which PBS finding is most characteristic of dysmegakaryopoiesis?
A) Oval macrocytes
B) Giant platelets and micromegakaryocytes
C) Hypochromic microcytes
D) Nuclear bridging

*Answer: B) Giant platelets and micromegakaryocytes

*Question: What is the MDS subtype treated specifically with Lenalidomide?
A) MDS-SF3B1
B) MDS-biTP53
C) MDS-5q
D) MDS-IB2

*Answer: C) MDS-5q

*Question: Which drug is approved for MDS with ring sideroblasts and MDS/MPN with ring sideroblasts and thrombocytosis?
A) Azacitidine
B) Decitabine
C) Lenalidomide
D) Luspatercept

*Answer: D) Luspatercept

*Question: Which of the following are the hypomethylating agents used in MDS treatment?
A) Lenalidomide and Luspatercept
B) Azacitidine and Decitabine
C) EPO and G-CSF
D) Thalidomide and Lenalidomide

*Answer: B) Azacitidine and Decitabine

*Question: What is the blast percentage in MDS-IB1 (Bone Marrow)?
A) 1–4%
B) 5–9%
C) 10–19%
D) 20–29%

*Answer: B) 5–9%

*Question: What blast percentage in bone marrow defines MDS-IB2?
A) 5–9%
B) 2–4%
C) 10–19%
D) 20–30%

*Answer: C) 10–19%

*Question: Which finding in MDS-IB2 can substitute for blast percentage in diagnosis?
A) Ring sideroblasts
B) Auer rods
C) Pseudo-Pelger Hüet anomaly
D) Giant platelets

*Answer: B) Auer rods

*Question: MDS-IB2 is associated with which clinical outcome?
A) Best prognosis
B) Stable long-term disease
C) Worst survival and highest AML transformation
D) Favorable response to Lenalidomide

*Answer: C) Worst survival and highest AML transformation

*Question: What is the main difference between MPN and MDS?
A) MPN has cytopenias; MDS has cytoses
B) MPN has cytoses (excess mature cells); MDS has cytopenias
C) MPN is acquired; MDS is inherited
D) MPN affects lymphoid cells; MDS affects myeloid cells

*Answer: B) MPN has cytoses (excess mature cells); MDS has cytopenias

*Question: What is the hallmark diagnostic criterion for CMML?
A) del(5q) with anemia
B) Persistent monocytosis >0.5 × 10⁹/L and >10% in PBS
C) Leukocytosis with BCR::ABL1 mutation
D) Ring sideroblasts >15% with thrombocytosis

*Answer: B) Persistent monocytosis >0.5 × 10⁹/L and >10% in PBS

*Question: What is the WBC cutoff that separates myelodysplastic CMML from myeloproliferative CMML?
A) 10 × 10⁹/L
B) 11 × 10⁹/L
C) 13 × 10⁹/L
D) 15 × 10⁹/L

*Answer: C) 13 × 10⁹/L

*Question: Which clinical symptoms are associated with myeloproliferative CMML?
A) Bruising, fatigue, recurrent infections
B) Bone pain, weight loss, splenomegaly
C) Oval macrocytes and ring sideroblasts
D) Giant platelets and bleeding

*Answer: B) Bone pain, weight loss, splenomegaly

*Question: MDS/MPN with Neutrophilia is also known as what?
A) Juvenile myelomonocytic leukemia
B) Atypical chronic myeloid leukemia
C) Chronic eosinophilic leukemia
D) Polycythemia vera

*Answer: B) Atypical chronic myeloid leukemia

*Question: Which mutation is ABSENT in MDS/MPN with Neutrophilia that differentiates it from classic CML?
A) JAK2
B) SF3B1
C) BCR::ABL1
D) TET2

*Answer: C) BCR::ABL1

*Question: What is the prognosis of MDS/MPN with Neutrophilia?
A) Most favorable of all MDS/MPN
B) Intermediate prognosis
C) Poor prognosis with high AML/BM failure transformation
D) Good prognosis with Lenalidomide treatment

*Answer: C) Poor prognosis with high AML/BM failure transformation

*Question: What is the thrombocyte count threshold for MDS/MPN-SF3B1-T diagnosis?
A) >150 × 10⁹/L
B) >300 × 10⁹/L
C) >450 × 10⁹/L
D) >600 × 10⁹/L

*Answer: C) >450 × 10⁹/L

*Question: Which concurrent mutation supports the diagnosis of MDS/MPN-SF3B1-T?
A) BCR::ABL1
B) TP53
C) JAK2
D) ASXL1

*Answer: C) JAK2

*Question: Which MDS/MPN subtype has the MOST favorable prognosis?
A) CMML myeloproliferative type
B) MDS/MPN with Neutrophilia
C) MDS/MPN-SF3B1-T
D) MDS/MPN NOS

*Answer: C) MDS/MPN-SF3B1-T

*Question: What percentage of MDS-SF3B1 cases show ring sideroblasts in bone marrow for WHO diagnosis?
A) ≥5%
B) ≥10%
C) ≥15%
D) ≥20%

*Answer: C) ≥15%

*Question: MDS-5q predominantly affects which demographic?
A) Elderly males
B) Young males
C) Predominantly females with median age 67
D) Children under 18

*Answer: C) Predominantly females with median age 67

*Question: What is the median survival for MDS-5q?
A) 12–24 months
B) 24–36 months
C) 54–146 months
D) 200+ months

*Answer: C) 54–146 months

*Question: Which of the following is NOT a feature of MDS-5q?
A) Anemia without other cytopenias
B) Dysplastic megakaryocytes
C) Auer rods present
D)

*Answer: C) Auer rods present

*Question: What characterizes MDS, Hypoplastic (h-MDS)?
A) Hypercellular marrow with multilineage dysplasia
B) Age-adjusted hypocellular bone marrow with cytopenia and dysplasia
C) Ring sideroblasts >15% with hypocellular marrow
D) Increased blasts with fibrosis

*Answer: B) Age-adjusted hypocellular bone marrow with cytopenia and dysplasia

*Question: What immune mechanism causes hypocellular marrow in h-MDS?
A) B cells producing anti-HSC antibodies
B) T cells targeting HSCs
C) NK cells destroying megakaryocytes
D) Neutrophil-mediated destruction of BM stroma

*Answer: B) T cells targeting HSCs

*Question: h-MDS must be differentiated from which two conditions?
A) CML and PNH
B) Aplastic anemia and PNH
C) AML and MPN
D) CMML and CML

*Answer: B) Aplastic anemia and PNH

*Question: What percentage of MDS cases does h-MDS account for?
A) 1–5%
B) 5–8%
C) 10–15%
D) 20–25%

*Answer: C) 10–15%

*Question: In the FAB classification, which subtype has the HIGHEST dyserythropoiesis (+++ rating)?
A) RARS
B) RAEB
C) RA/RC
D) CMML

*Answer: C) RA/RC

*Question: In FAB classification, which subtype has WBC count that is INCREASED?
A) RA
B) RARS
C) RAEB
D) CMML

*Answer: D) CMML

*Question: In FAB classification, what is the blast percentage in bone marrow for RAEBIT?
A)

*Answer: C) 20–30%

*Question: In FAB classification, RARS requires ringed sideroblasts of greater than what percentage?
A) 5%
B) 10%
C) 15%
D) 20%

*Answer: C) 15%

*Question: Which of the following inherited BM failure syndromes predisposes to MDS?
A) Sickle cell disease
B) Fanconi anemia
C) Hemophilia A
D) Von Willebrand disease

*Answer: B) Fanconi anemia

*Question: Which of the following is NOT included in the 5 major groups of gene mutations in MDS?
A) RNA Splicing
B) DNA Methylation
C) Telomere Shortening
D) Chromatin Modifiers

*Answer: C) Telomere Shortening

*Question: Which mutation in MDS is associated with DNA methylation?
A) SF3B1
B) SRSF2
C) DNMT3A
D) RUNX1

*Answer: C) DNMT3A

*Question: Which of the following best describes the paradox of MDS?
A) Cytopenia with hypocellular bone marrow
B) Cytopenia despite hypercellular bone marrow
C) Cytosis with hypercellular bone marrow
D) Cytosis despite hypocellular bone marrow

*Answer: B) Cytopenia despite hypercellular bone marrow

*Question: Which BM finding in dyserythropoiesis involves connections between two erythroid precursor nuclei?
A) Nuclear budding
B) Internuclear bridging
C) Nuclear fragmentation
D) Megaloblastoid development

*Answer: B) Internuclear bridging

*Question: What stain is used to identify ring sideroblasts?
A) Wright-Giemsa
B) PAS stain
C) Prussian Blue
D) Sudan Black B

*Answer: C) Prussian Blue

*Question: What is the hallmark PBS finding of dysmyelopoiesis that represents nuclear-cytoplasmic asynchrony?
A) Oval macrocytes
B) Persistence of cytoplasmic basophilia in otherwise mature WBCs
C) Dimorphic RBC population
D) Giant platelets

*Answer: B) Persistence of cytoplasmic basophilia in otherwise mature WBCs

*Question: What is the peripheral blood monocyte count threshold (absolute) required for CMML diagnosis?
A) >0.1 × 10⁹/L
B) >0.3 × 10⁹/L
C) >0.5 × 10⁹/L
D) >1.0 × 10⁹/L

*Answer: C) >0.5 × 10⁹/L

*Question: In CMML, blasts and promonocytes in PBS and BM must be less than what percentage?
A) 10%
B) 15%
C) 20%
D) 25%

*Answer: C) 20%

*Question: Which of the following is a common mutation seen in CMML?
A) BCR::ABL1
B) JAK2 V617F
C) TET2, SRSF2, ASXL1, RUNX1
D) SF3B1 only

*Answer: C) TET2, SRSF2, ASXL1, RUNX1

*Question: What is the International Consensus Classification (ICC 2022) equivalent of MDS/MPN with Neutrophilia?
A) Juvenile myelomonocytic leukemia
B) Atypical chronic myeloid leukemia (aCML)
C) Chronic neutrophilic leukemia
D) MDS/MPN NOS

*Answer: B) Atypical chronic myeloid leukemia (aCML)

*Question: Which childhood MDS subtype accounts for 80% of cMDS-LB cases?
A) cMDS-LB NOS
B) cMDS-LB hypercellular
C) cMDS-LB hypocellular
D) cMDS-IB

*Answer: C) cMDS-LB hypocellular

*Question: Which pathway mutation is characteristic of childhood MDS with increased blasts?
A) TP53 pathway
B) RAS pathway
C) JAK-STAT pathway
D) WNT pathway

*Answer: B) RAS pathway

*Question: Which of the following is in the differential diagnosis of childhood MDS with increased blasts?
A) Aplastic anemia
B) PNH
C) Down syndrome and JMMoL
D) Fanconi anemia only

*Answer: C) Down syndrome and JMMoL

*Question: What BM blast range defines Childhood MDS with Increased Blasts (cMDS-IB)?
A)

*Answer: C) 5–19% BM

*Question: Which of the following is true about MDS-biTP53?
A) Single copy of TP53 is mutated
B) Only deletion of TP53 is required
C) Both copies of TP53 are mutated or one is mutated and the other deleted
D) TP53 mutation is associated with favorable prognosis

*Answer: C) Both copies of TP53 are mutated or one is mutated and the other deleted

*Question: What percentage of de novo MDS does MDS-biTP53 account for?
A) 1–2%
B) 5–10%
C) 15–20%
D) 25–30%

*Answer: B) 5–10%

*Question: Which of the following conditions is NOT in the differential diagnosis of MDS?
A) Paroxysmal nocturnal hemoglobinuria
B) Copper deficiency
C) Multiple myeloma
D) Parvovirus B19

*Answer: C) Multiple myeloma

*Question: In MDS, erythrocytes show decreased response to which growth factor?
A) G-CSF
B) TPO
C) Erythropoietin (EPO)
D) GM-CSF

*Answer: C) Erythropoietin (EPO)

*Question: Platelet dysfunction in MDS causes bleeding despite adequate platelet count, along with what other abnormality?
A) Thrombocytopenia
B) Hyperaggregation
C) Decreased vWF
D) Prolonged clotting time only

*Answer: B) Hyperaggregation

*Question: Which granulocyte enzyme is decreased in MDS contributing to impaired microbicidal capacity?
A) Amylase and lipase
B) Myeloperoxidase (MPO) and Alkaline Phosphatase (ALP)
C) Lactoferrin and lysozyme
D) Catalase and peroxidase

*Answer: B) Myeloperoxidase (MPO) and Alkaline Phosphatase (ALP)

*Question: What is the definition of dysplasia in MDS?
A) Increased production of abnormal clonal cells
B) Presence of abnormal cells within a tissue or organ
C) Death of cells due to inflammatory pathways
D) Clonal expansion of lymphoid precursors

*Answer: B) Presence of abnormal cells within a tissue or organ

*Question: What does the prefix "DYS" mean in myelodysplastic?
A) Formation
B) Myeloid cell lineage
C) Abnormality in morphology and/or function
D) Destruction

*Answer: C) Abnormality in morphology and/or function

*Question: The International Consensus Classification (ICC 2022) created which new blast category?
A) MDS/AML (10–19% blasts)
B) MDS-IB3 (20–25% blasts)
C) MDS-LB3 (15–19% blasts)
D) MDS/CML (5–9% blasts)

*Answer: A) MDS/AML (10–19% blasts)

*Question: Which PBS finding in dyserythropoiesis occurs despite adequate iron stores?
A) Oval macrocytes
B) Hypochromic microcytes
C) Dimorphic RBCs
D) Basophilic stippling

*Answer: B) Hypochromic microcytes

*Question: What BM finding in dyserythropoiesis mimics vitamin B12/folate deficiency but occurs with normal levels?
A) Ring sideroblasts
B) Megaloblastoid development
C) Nuclear bridging
D) Erythrocytic hyperplasia

*Answer: B) Megaloblastoid development

*Question: Which nuclear abnormality is seen in neutrophils in dysmyelopoiesis?
A) Nuclear bridging
B) Bilobed nucleus only
C) Ring-shaped nuclei
D) Multiple round nuclei

*Answer: C) Ring-shaped nuclei

*Question: In dysmegakaryopoiesis, what is the name for small-sized abnormal megakaryocytes?
A) Micromegakaryoblasts only
B) Hypomegakaryocytes
C) Micromegakaryocytes
D) Nanomegakaryocytes

*Answer: C) Micromegakaryocytes

*Question: MDS with Low Blasts (MDS-LB) requires blasts to be less than what in the peripheral blood?
A)

*Answer: B)

*Question: Which classification system uses FIVE classes based on morphology, amount of dysplasia, and number of blasts?
A) WHO 2022
B) ICC 2022
C) FAB classification
D) Revised IPSS

*Answer: C) FAB classification

*Question: In WHO 2022 classification, which category preserved the name Myelodysplastic Syndromes?
A) WHO classification
B) FAB classification
C) International Consensus Classification (ICC 2022)
D) Revised IPSS classification

*Answer: C) International Consensus Classification (ICC 2022)

*Question: Which MDS subtype requires an age-adjusted hypocellular marrow for diagnosis?
A) MDS-LB multilineage
B) MDS-IB1
C) MDS, Hypoplastic (h-MDS)
D) MDS-5q

*Answer: C) MDS, Hypoplastic (h-MDS)

*Question: What is the key distinguishing feature of MDS-LB Multilineage Dysplasia compared to Single Lineage Dysplasia?
A) Higher blast count
B) Dysplasia in 2 or 3 cell lineages with worse prognosis
C) Presence of ring sideroblasts
D) Lower blast count with better prognosis

*Answer: B) Dysplasia in 2 or 3 cell lineages with worse prognosis

*Question: In MDS, which erythrocyte abnormality results from the shortened survival of red blood cells?
A) Increased response to EPO
B) Oval macrocytosis
C) Shortened survival contributing to anemia
D) Hyperchromia

*Answer: C) Shortened survival contributing to anemia

*Question: Which of the following is true about the diagnostic workup for MDS?
A) Bone marrow examination alone is sufficient
B) Somatic mutation analysis alone confirms diagnosis
C) Workup includes BM morphology, karyotype, flow cytometry, and somatic mutation analysis
D) PBS findings alone are diagnostic

*Answer: C) Workup includes BM morphology, karyotype, flow cytometry, and somatic mutation analysis

*Question: Which of the following MDS/MPN subtypes is characterized by features of BOTH cytopenia AND cytosis simultaneously?
A) MDS-IB2
B) MDS-biTP53
C) MDS/MPN overlap neoplasms
D) MDS-h

*Answer: C) MDS/MPN overlap neoplasms

*Question: Childhood MDS with Low Blasts Hypocellular subtype accounts for what percentage of cMDS-LB?
A) 50%
B) 60%
C) 70%
D) 80%

*Answer: D) 80%

*Question: What distinguishes MDS from MPN in terms of cell production?
A) MDS overproduces mature cells; MPN shows ineffective hematopoiesis
B) MDS shows ineffective hematopoiesis with cytopenias; MPN overproduces mature cells
C) Both show the same level of hematopoiesis
D) MPN has cytopenias; MDS has cytoses

*Answer: B) MDS shows ineffective hematopoiesis with cytopenias; MPN overproduces mature cells

*Question: In MDS with Increased Blasts, what percentage of cases does MDS-IB represent among all MDS?
A) 5–10%
B) 15–20%
C) 28–39%
D) 45–55%

*Answer: C) 28–39%

*Question: Which of the following correctly describes the BM blast range for MDS with Fibrosis?
A)

*Answer: B) 5–19% BM blasts with 2–19% PB blasts

*Question: Which low-risk MDS supportive therapy agent stimulates platelet production?
A) EPO
B) G-CSF
C) TPO
D) Iron chelators

*Answer: C) TPO

*Question: Which of the following is the most common overall mutation found in MDS?
A) SF3B1
B) TP53
C) TET2
D) RUNX1

*Answer: C) TET2

*Question: Luspatercept is approved for which specific MDS condition?
A) MDS-biTP53
B) MDS with ring sideroblasts and MDS/MPN with ring sideroblasts and thrombocytosis
C) MDS-5q only
D) MDS-IB2

*Answer: B) MDS with ring sideroblasts and MDS/MPN with ring sideroblasts and thrombocytosis

*Question: Which of the following MDS inherited syndromes is associated with exocrine pancreatic insufficiency?
A) Diamond-Blackfan anemia
B) Fanconi anemia
C) Shwachman-Diamond syndrome
D) Dyskeratosis congenita

*Answer: C) Shwachman-Diamond syndrome

*Question: What is the PB blast percentage range for MDS-IB1?
A)

*Answer: B) 2–4%

*Question: Which of the following correctly pairs an MDS subtype with its specific treatment?
A) MDS-biTP53 — Lenalidomide
B) MDS-SF3B1 — Azacitidine only
C) MDS-5q — Lenalidomide
D) MDS-IB2 — Luspatercept

*Answer: C) MDS-5q — Lenalidomide

*Question: What is the key difference between RAEB and RAEBIT in FAB classification regarding PB blasts?
A) RAEB has >5% PB blasts; RAEBIT has

*Answer: B) RAEB has

*Question: Which of the following best describes the MDS/MPN-SF3B1-T minimum ring sideroblast percentage?
A) >5%
B) >10%
C) >15%
D) >20%

*Answer: C) >15%

*Question: In the diagnostic workup for MDS, what does a non-clonal cytopenia result lead to?
A) Diagnosis of MDS
B) Classification as CCUS
C) Non-malignant/secondary etiology or other malignant cause
D) Classification as CHIP

*Answer: C) Non-malignant/secondary etiology or other malignant cause