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7 Cards in this Set
- Front
- Back
Myeloproliferative Disorders
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Myeloproliferative Disorders
clonal stem cell disorders associated with abnormal proliferation of mature myeloid elements 1. Polycythemia Vera 2. Essential Thrombocytosis 3. Chronic Myelogenous Leukemia |
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Presentation
- Bone marrow failure • Anemia – fatigue, weakness, shortness of breath • Thrombocytopenia – increased bruising and bleeding • Leukopenia/neutropenia – infections - Disseminated Intravascular Coagulation - Confusion |
Acute Myelogenous Leukemia(AML)
Epidemiology - Incidence – 2.5/100,000/year – Median age – 63 years – 80-90% of adult leukemias – Causes include ionizing radiation, organic solvents such as benzene, previous chemotherapy, history of myelodysplastic syndrome (MDS), and inherited disorders of DNA repair (Down’s, Fanconi’s anemia, Wiskott-Aldrich), though most cases do not have precipitating cause Pathology - Acute Leukemia is a cancer of the bone marrow progenitors (progenitors are immature marrow cells still capable of division) - Myeloblasts (immature myeloid cells) proliferate and are unable to mature into normal cells - These blasts eventually dominate the bone marrow, suppressing normal hematopoiesis – therefore decreases red cell, platelet, and neutrophil production - Classically, eight subtypes of AML(the French-American-British-FAB-classification system), however more recently divided into more categories - Numerous known chromosomal (cytogenetic) abnormalities – some even associated with good prognosis o 15:17 translocation – found in Acute Promyelocytic Leukemia (also known as AML-M3)- this subtype of AML has the best prognosis >70% long term survival o 8;21 translocation (found in AML-M2) and inversion 16(found in AML-M4Eo) also associated with better prognosis - By definition acute leukemia must have 20% leukemic blasts in the bone marrow |
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Presentation - Bone marrow failure
• Anemia – fatigue, weakness, shortness of breath • Thrombocytopenia – increased bruising and bleeding • Leukopenia/neutrapenia – infections - No confusion |
Myelodysplastic Syndrome (MDS)
Epidemiology - Disease of the elderly - Occurs in about 1 out of 500 persons between 60-75 - Most cases idiopathic, but known causes include ionizing radiation, organic solvents such as benzene, and previous chemotherapy – note these are the same as AML Pathology - A clonal disorder characterized by ineffective and disordered hematopoiesis - Abnormal clone suppresses normal maturation of the other cell lines resulting in reduced production of red cells, myeloid cells and platelets - Typically characterized by chromosomal(cytogenetic) abnormalities |
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- Headaches, “ruddy” complexion, visual changes, neuro symptoms from hyperviscosity of elevated red count
- Stroke or heart attack |
Polycythemia Vera
Epidemiology - Occurs in 1-3/100,000 people - Median age of diagnosis is 65 Pathology - Disorder causes elevated red cell count, though often platelet count and white count are elevated as well - High red cell count causes hyperviscosity, leading to decreased oxygen delivery, and therefore poor CNS function - Combination of high red cell count and high platelet count increases substantially the risk of thrombotic episode such as a stroke or heart attack - Can convert to AML – but rare - Associated with a genetic mutation in the Janus Kinase 2 gene – the JAK2 V617F mutation – identifying this gene in the context of erythrocytosis establishes the diagnosis |
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Pathology
- Clonal stem cell disorder that causes increase platelet production - High platelet count increases risk for thrombosis and/or bleeding complications - Very rarely transforms into AML - Can be associated with the JAK2 mutation |
Essential Thrombocytosis
Epidemiology - Less common than P.Vera - Average age of diagnosis is 60-65 - Up to 25% are less than age 40 Presentation - Often patients are asymptomatic and found on routine blood work - Thrombotic event – such as stroke or heart attack |
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Chronic Myelogenous Leukemia
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Chronic Myelogenous Leukemia
Chronic Myelogenous Leukemia Epidemiology - Occurs in 1/100,000 people - Median age is 43, but can develop at any age Pathology - Proliferation of maturing myeloid elements - Characterized by the Philadelphia Chromosome (9;22 translocation) – this translocation connects bcr and abl (known as the bcr-abl translocation) – Identifying this translocation makes the diagnosis! - This translocation permanently turns on a tyrosine kinase that induces proliferation of the abnormal stem cell clone - Typically, white cells, red cells and platelet counts are elevated - Will always progress to AML (called the blast phase of CML) - this type of AML is not cured with conventional therapy Presentation - Patients can be asymptomatic - Weakness, fatigue from high white count due to increase cytokine release |
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AML 1-7
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AML 1-7:
AML 1- 3: AMLs of granulocytes AML 4-5: AMLs of monoctyes AML 6 : AML of eythrocytes AML 7: AML of megakaryocytes |