Ch19 Myelodysplastic Syndromes Flashcards
The median age of MDS is:
70
What is a heterogeneous group of clonal hematological malignancies characterized by cytopenias, dysplasia blood cells, and a propensity to transform into acute leukemia
Myelodysplastic syndrome (MDS)
What is the hallmark of MDS?
Monoclonal derivation of cells
What is the cell of origin in MDS
A pluripotent stem cell which is already committed to myeloid differentiation
Programmed cell death is known as what?
Apoptosis
What are the 3 stages of apoptosis
Initiation, commitment, execution
How can apoptosis be initiated?
By chemotherapy, radiotherapy, corticosteroid, and growth factors
MDS patients have an _______ rate of apoptosis
Increased
MDS has an imbalance between growth-stimulatory and growth inhibitory cytokines that causes:
An increase in apoptosis
What is the most common genetic anomaly in MDS?
Loss of genetic material - chromosomal deletion
Phenotypical changes of MDS are:
Number of blast cells increase
Leukocyte sis may replace leukopenia
Enlarged spleen and liver
Proliferation of abnormal cells
What type of anemia is present in at least 90% of MDS cases?
Macrocytic or normocytric
In MDS RBC may have:
Pyruvate kinase deficiency and increased levels of HbF
The survival rate of patients with MDS is:
Less than 2 years
What is the only potential cure of MDS?
Allogeneic stem cell transplantation
What type of anemia is most common in MDS?
Macrocytic and normochromic
Which morphological abnormalities are helpful in the diagnosis of MDS?
Pseudo-pelger-Huët and hypogranulation
Ringed sideroblasts
Micromegakaryocytes, monolobular, megakaryocyte
What cell surface antigens is of prognostic significance in MDS?
CD34
What MDS subgroup has the most favorable prognosis?
RARS
Which type of treatment may offer a cure in patients with MDS
Allogeneic BM transplantation
What agent may lead to secondary MDS?
Alkylating agents
What is most likely to affect the prognosis in MDS?
Increased BM myeloblasts
What chromosomal abnormality is most often found in female patients with RA and elevated platelet counts?
5q-
What biologic or genetic anomalies are important in the pathogenesis of MDS?
Chromosomal deletion a