Myelodysplasgic Syndromes And Acute Myeloid Leukemia Flashcards
What is the result of MDS and AML?
Decreased production of one or more of the major hematopoitic lineages
What are clonal stem cell disorders?
Stem cells make same cells
What is the halmark ?
Bone marrow failure
What is MDS?
Heterogeneous hematopoisis and increased apoptosis with periferial cytopenia
What about MDS?
Recurrent genetic abnormailitis
Baraible increase in blast cell percenatge
Increase risk of developing acute myeloid leukemai pre leukemic
Mostly 70
What is the pathogensis of MDS ?
Genetic disorders in myeloid progenitor or in blast cells
That is why it can affect one type or many because progenitor
What is etiology ? Of MDS
Primary denovo
Secondary chemical benzene smoking chemotherapy radiation
How diagnose mds ?
Clinical features Cytopenia and severity Laboratory features CBC Cytopenia Elevated MCV ( macrocytic the anemai )
Peripharal blood
Dysplastic feautres in one or more cell lines
+- circulating blast cells
Bone marrow
Dysplastic features in one or more cell lines
+- increased % of blasts cells
Define Acute myeloid leukemia ? AML
Uncontrolled clonal proliferation and accumulation of immmature myeloid ( myeloblasts ) in bone marrow also blood or tissue skin lung u name causing bone marrow failure
Aml is
Hetergenous depend on type some form we can cure others we cant
20% of childhood less than 15 years acute leukemia
In adults 80 myloid ans rest lymphoid in adults more than 15
What cause acute myeloid leukemia ?
Ionizing radiation
Chemicals benzene and other petroleum derivatives
Chemotherapy
Alkylating ( secondary AML )
Germline predisposition
Inheried or de nove grnetic defects like down syndroem
What is the pathogensis ?
Two hit model of leukemigesis ?
- Loss of function of transcription needed for differentiation
Lack of differntiation ( loss of function
2. Gain of function mutation of tyrosine kineses Will cause enzyme active ( tyrosine important for phosphorylation leading to abnormal proliferation Enhanced proliferation ( gain of function
What is the clinical presentation of AML ?
Due to bone marrow replacement failure related cytopenia
Due to extramedullary infilteration
Hepatospleenomegaly
Skin lesions
Gum hypertrophy
Due to release of procoagulanta or profibrinolytic substance s
DIC
Excessive firinoluis
How to diagnose ?
Morphology
Amust
Bone marrow aspirate smears
Identifiction of a variety of blast cellls
Auer rods ( in acute leukemia for sure acute myeold anmie but not see it ok )
Should find more than 20 percent blast cells in the bone marrow
Immunophenotyping immune antibodies to identify which type
Flow cytometric analysis using monoclonal antobodies
Rapid and sensitive
Detect specific cellular antigens surface or intracellur
Not on linlinage but also which one ehich origen
Cytogenetic and genotyping