Myelodysplasia

Question 1

Disorders of hematopoietic stem cells characterized by bone marrow failure and abnormalities of cells in peripheral blood

Answer 1

Myelodysplasia

Question 2

Hallmark of myelodysplasia

Answer 2

Simultaneous proliferation and apoptosis of hematopoietic cells (ineffective hematopoiesis) leading to hypercellular bone marrow but pancytopenia in peripheral blood

Question 3

Patogenesis of myelodysplasia

Answer 3

Genetic damage to a mkultipotent hematopoietic progenitor cell

Question 4

Dysplasia of only red cells…

Answer 4

Refractory anemia

Increased blast cells in blood or bone marrow

Question 5

Dysplasia of neutrophils or platelets or 2 or more myeloid lineages

Answer 5

Refractory cytopenia with multilineaaage dysplasia (RCMD)

Question 6

What are ring sideroblasts?

Answer 6

Erythroid precursor with 5 or more iron granules encircling at least 1/3 of the nucleus

Appearance of ring sideroblasts is caused by iron deposition in the mito

Question 7

Anemic disorder classified as a myelodysplastic-myeloproliferative disease

Answer 7

Refractory anemia with ring sideroblasts and thrombocytosis

Question 8

Incidence of myelodysplasia

Answer 8

4 in 100 000

Question 9

Myelodysplasia is more common in which sex?

Answer 9

Slight male predominance

Question 10

Age of patients with myelodysplasia

Answer 10

Half of patients are older than 70 years and less than 25% are less than 50 years

Question 11

Evolution of myelodysplasia is ….

Answer 11

Slow

Question 12

Symptoms of myelodysplasia, although not always present may be:

Answer 12

• Those of anemia
• Those of infection
• Easy bruising or bleeding

Question 13

Differential diagnostics of myelodysplasia, which show dysplastic features in bone marrow may be:

Answer 13

• Excess alcohol intake
• Megaloblastic anemia
• Parvovirus
• Recovery from cytotoxic chemoteraphy and G-CSF therapy

Question 14

How can we confirm an MDS diagnosis

Answer 14

10% of cells in a lineage should de dysplastic

Question 15

Although hypercellularity of the bone marrow is characteristic of myelodysplasia, in 20% of cases..

Answer 15

Bone marrow is hypocellular and may ressemble aplastic anemia

Question 16

Laboratory findings in peripheral blood of patients with myelodysplasia may include:

Answer 16

• Pancytopenia
• Red cells may be macrocytic, dimorphic or hypochromic
• Normoblasts may be present
• Low reticulocyte count
• Reduced granaulocytes with lack of granulation
• Pelger abnormality
• Platelets may be large or small and decreased or increased

Pelger-Huet anomaly (PHA) is an inherited blood condition in which the nuclei of several types of white blood cells (neutrophils and eosinophils) have unusual shape (bilobed, peanut or dumbbell-shaped instead of the normal trilobed shape) and unusual structure (coarse and lumpy)

Question 17

Laboratory findings in bone marrow of patients with myelodysplasia may include:

Answer 17

• Increased cellularity
• Multinucleate normoblasts
• Ring sideroblasts
• Granulocyte precursors with defective granulation
• Abnormal megakaryocytes

Question 18

Prognosis associated with augmented blasts in blood

Answer 18

Poor prognosis

Laboratory findings oof peripheral blood in MDS patients