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Myelodysplastic Syndromes and
24 Myelodysplastic/Myeloproliferative
CHAPTER
Neoplasms
KEY TERMS
chronic m yelom onocytic leukem ia myelodysplastic syndromes (MDSs) refractory anem ia with excess blasts
idiopathic cytopenia of undeterm ined myeloproliferative neoplasm s (RAEB-1 and RAEB-2)
signi cance (ICUS) (MPNs) refractory anem ia with ring sidero-
internuclear bridging (INB) refractory anem ia (RA) blasts (RARSs)
LEARNING OUTCOMES
Classi cation ■ Compare the laboratory features of speci c types of MDSs.
■ Describe the comparative characteristics of the French-American- ■ Distinguish between agranular blasts, granular blasts, and
British (FAB) and World Health Organization (WHO) classi cation of promyelocytes.
myelodysplastic syndromes (MDSs) and myelodysplastic/myelopro- ■ Calculate the percentage of myeloblasts in the bone marrow.
liferative neoplasms (MDS/MPN). Myelodysplastic syndromes/ myeloproliferative
Pathophysiology neoplasms
Explain the pathophysiology of MDSs. ■ Describe the unique features and laboratory characteristics of
chronic myelomonocytic leukemia (CMML).
Etiology Distinguish between MDS and acute leukemia
■ Explain the causes or predisposing factors of primary and secondary
MDSs. Treatment
Explain the forms of treatment and supportive care for the MDSs.
Epidemiology
■ Describe the age and gender distribution of MDSs. Relationship of cytogenetics to prognosis
■ Discuss factors that can affect prognosis in the MDSs, including FAB
Chromosomal abnormalities classi cation and karyotype.
■ Brie y describe the causes, types, and consequences of chromo- Compare the parameters used in the International Scale for
somal abnormalities in MDSs. Prognosis.
■ List the incidence of chromosomal abnormalities.
■ Describe the relationship of cytogenetic ndings to prognosis in Case studies
MDSs. Analyze the patient history, clinical signs and symptoms, and labo-
ratory data for the stated case studies, answer the related critical
Clinical signs and symptoms thinking questions, and conclude the most likely diagnosis.
■ Explain the clinical signs and symptoms of MDSs.
Laboratory manifestations
■ Itemize the cellular alterations, with an emphasis on the prominent NOTE:
Indicates MLT and MLS core content
■
features and additional hematological features in the four sub- Indicates MLT (optional) and MLS advanced content
groups of MDS/MPN.
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