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Thrombocytosis Essential and Reactive PowerPoint Presentation

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Description : This is a powerpoint presentation of thrombocytosis disorder

Tags : Thrombocytosis

Published on : Mar 18, 2019 | Views : 2153 | Downloads : 4

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Slide 1-

Thrombocytosis Essential and Reactive Under supervision of Prof. Dr/ Amr Gawaly
Slide 2-

Stem Cell Basis of Hematopoiesis Normal Myeloproliferative disease Stem cells Progenitors Blood
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Essential Thrombocythaemia (ET) Clonal MPD Persistent elevation of Platelet >600 x109/L Poorly understood Lack of positive diagnostic criteria 2.5 cases/100000 M:F 2:1 Median age at diagnosis: 60, however 20% cases <40yrs
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Clinical Features Vasomotor Headache Lightheadedness Syncope Erythromelalgia (burning pain of the hands or feet associated with erythema and warmth) Transient visual disturbances (eg, amaurosis fujax, scintillating scotomata, ocular migraine) Thrombosis and Haemorrhage Transformation
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Investigations ET is a diagnosis of exclusion Rule out other causes of elevated platelet count
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Diagnostic criteria for ET Platelet count >600 x 109/L for at least 2 months Megakaryocytic hyperplasia on bone marrow aspiration and biopsy No cause for reactive thrombocytosis Absence of the Philadelphia chromosome Normal red blood cell (RBC) mass or a HCT <0.48 Presence of stainable iron in a bone marrow aspiration No evidence of myelofibrosis No evidence of MDS
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platelets
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Excess megakaryocytes “hyperplasia’
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Therapy of ET based on the risk of thrombosis
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Myelodysplastic Syndrome “brief overview”
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Intro MDS comprises a group of malignant stem cell disorders characterized by ineffective blood cell production and variable risk of transformation to acute leukemia.
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Prevalence ~ 10,000 cases annually in US 4.1 MDS / 2.1 AML per 100,000 Risk of Development increases w/ Age Unusual <50yrs, unless tx induced Median age 65 or greater, w/ male predominance
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Predisposing Elements Heritable – Genetic Disorders including Down’s, Congential Neutropenias, Fanconi’s Anemia, Ataxia Telangiectasia, NF-1…. Acquired – Mutagen Exposure (alkylators, Topio II, Radioactive), Hemapoietic cell xplants, environmental exposures, Aplastic Anemia, PNH, Polycythemia Vera
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MDS Etiology Two etiologic categories of MDS: 1.) De Novo: Associated with: -benzene exposure (gasoline) -cigarettesmoking -viruses -Fanconi’s anemia 2.) Therapy related: Associated with: -alkylating agent chemotherapy -radiation
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MDS – Clinical Symptoms Ecchymoses Fatigue Pallor Ecchymoses/petechiae Abnormal bleeding Infection
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Laboratory Bone Marrow and Blood changes variable, divided into FAB MDS subtypes. Chromosomal Abnormalities are associated as well. Anemia almost always present w/ low retic response Pancytopenia in up to 50% of cases <5% have isolated neutropenia or thrombocytopenia w/o anemia
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MDS subtypes: FAB classification Refractory Anemia 21% (RA) RARS (refractory anemia w/ ringed sideroblasts 17%) RA w/ excess blasts 37% (RAEB) CMML (chronic myelomonoctic leukemia) 13%
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Ringed Sideroblasts
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Thank You!