Myelodysplastic sundrome Flashcards
Myelodysplastic syndrome
The myelodysplastic syndromes (MDS) comprise a heterogeneous group of malignant hematopoietic stem cell disorders characterized by dysplastic and ineffective blood cell production and a variable risk of transformation to acute leukemia.
MDS occurs most commonly in older adults, with a median age at diagnosis in most series of ≥65 years and a male predominance
clinical presentation of MDS
Anemia is the most common cytopenia and can manifest as fatigue, weakness, exercise intolerance, angina, dizziness
MDS may develop infections related to neutropenia and granulocyte dysfunction
cellular characteristics of MDS
MDS is characterized by abnormal cell morphology (dysplasia) and quantitative changes in one or more of the blood and bone marrow elements
macrocytic or normocytic anemia; neutropenia and thrombocytopenia are more variable. Pancytopenia (ie, anemia, leukopenia, and thrombocytopenia) is present at the time of diagnosis in up to 50 percent of patients
hypercellular bone marrow, immature cells
Diagnosis of MDS
otherwise unexplained quantitative changes in one or more of the blood and bone marrow elements
Morphologic evidence of significant dysplasia
Blast forms account for less than 20 percent of the total nucleated cells of the bone marrow aspirate and peripheral blood
higher = AML
Asymptomatic MDS treatment
managed expectantly
vaccinations up to date
smoking cessation
Symptomatic MDS treatment
goal is to relieve symptoms and prevent transformation to AML
Supportive care includes the use of antibiotics for infection and red cell and platelet transfusions
Low intensity therapies include hematopoietic growth factors, azacitidine and decitabine (often referred to as hypomethylating agents), immunosuppressive therapy, and lenalidomide
combination chemotherapy or allogeneic HCT in an attempt to alter the disease course.
