Lecture 17 - Clonal Marrow Disorders

Lecture 17 - Clonal Marrow Disorders - Lecture 17 Clonal...

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Lecture 17 – Clonal Marrow Disorders Introduction: Acquired genetic changes in marrow progenitor cells that result in variable clinical categories of disease: (in hematopoietic stem cells) Myelodysplastic syndromes: hypercellular marrow with cytopenias (ineffective hematopoiesis) most patients will present with anemia , pancytopenia the marrow is cellular but the cell is not maturing normally and not getting out of the marrow; the precursor are dying before they can get out to circulation) Myeloproliferative neoplasms: hypercellular marrow with increased blood counts Producing too many cells in marrow and getting out into the blood Very high blood counts Note sometimes as the disease progresses the blood count can decrease this is when it is hard to distinguish between the myelodysplastic or myeloproliferative Neoplastic cells that have all the stages of maturation it will almost have normal cells just too many of them! Acute Leukemia: greater than 20% blasts in marrow Overproduction of the less mature forms Neoplastic hematopoietic precursors have variable self-renewal vs maturation resulting in different pathology - Totipotenet: More self-renewal; minimal differentiation - Differentiated cells: no self-renewal and terminally differentiated SUMMARY OF MDS MDS is a clinical, pathologic, and cytogenetic diagnosis Cytogenetics, blast percentage, and cytopenias are best prognostic features Cytopenias cause most morbidity mortality. Minority progress to AML Treatment now is more than supportive *****MDS****** Pseudo-pelger huet segmented neutrophil o Neutrophils that are Bi-loped, occasionally uni-loped (pelger huet) not 1/2 o Pseudo = acquired , because there are inherited genes that are involved with the # of lopes on the neutrophils o There are certain drugs immunosuppressive (drugs for organ transplant, anti viral) idiosyncratic neutrophil hyposegmentation - Hypogranular seg: no granular on the neutrophils - Blasts : Circulating blast good finding that the person has marrow disorder [ normally people don’t have blasts circulating in blood , unless person on growth factors] o Middle = nucleolus Azacytidine and MDS **There is certain genes that are heypermethylatin that shuts down the genes First FDA approved drug for MDS De-methylating agent for DNA Hypermethylated genes include: P15cyclin-dependent kinase inhibitor RARbeta, E-cadherin, fragile histidine triad Improvement occurs in 60+%, CR 6% Toxicity mild, mostly cytopenias It doesn’t affect the survival for the high risk significantly IPSS - Marrow blast % o higher blast higher score - Cytopenias o 1 = IPSS 0 o 2 or 3 = IPSS 0.5 - Cytogenetic features o Karyotype (good = 0, med = 0.5 poor = 1.0) o Good: normal, -Y, 5q-, 20q- (70%) o Intermediate: Not good or poor (14%)
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o Poor: complex (>3 abnormalities) or chromosome 7 abnormalities (16%) - Survival o higher survival
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Lecture 17 - Clonal Marrow Disorders - Lecture 17 Clonal...

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