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7 Cards in this Set

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Myeloproliferative Disorders
Myeloproliferative Disorders

clonal stem cell disorders associated with abnormal proliferation of mature myeloid elements

1. Polycythemia Vera
2. Essential Thrombocytosis
3. Chronic Myelogenous Leukemia
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
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
- 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
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
Chronic Myelogenous Leukemia
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
AML 1-7
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