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Myeloproliferative Disorders
Hematology
9
Biology
Not Applicable
11/28/2011

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Term
All MPD's
Definition
Can be pre-leukemic states
Increased production of all cell lines
Increased RBC's, WBC's, and Platelets in the bone marrow
Decreased RBC's, WBC's, and Platelets in the peripheral blood
Increase in defective stem cells
Abnormal cells overtake the bone marrow and shut down hematopoiesis
G6PD enzymes become heinz bodies
Hemorrhage, thrombosis, infections, anemia, bizarre platelets
Bone marrow has decreased iron stores, but normal serum iron and ferritin levels
Hypercellular bone marrow becomes fibrotic
Term
Chronic Myelocytic Leukemia (CML)
Definition
aka Chronic Granulocytic Leukemia
aka Chronic Myeloid Leukemia
3 Phases - Chronic, Accelerated, Blast Crisis
Philadelphia Chromosome
Most common MPD
Chloromas
Leukocytosis (WBC: >100)
Thrombocytosis
Normocytic, normochromic anemia
Pseudo-pelger-huets
Decreased LAP score
Increased M:E ratio
Auer Rods
NRBC's
Retic count normal to increased
Term
Juvenile CML
Definition
Affects 10-14 year olds
WBC Count: 30
<10% blasts
LAP score is normal to increased
Resistant to chemotherapy
Term
Chronic Eosinophilic Leukemia
Definition
Seen in middle-aged males
30-70% of the WBC's are eosinophils
Term
Chronic Basophilic Leukemia
Definition
Seen in middle-aged males
40-80% of the WBC's are basophils
Term
Chronic Neutrophilic Leukemia
Definition
Seen in people over 50 years of age
Increased LAP score
Diagnosed by Exclusion
Term
Myelofibrosis with Myeloid Metaplasia (MMM)
Definition
aka Chronic Idiopathic Myelofibrosis
Caused by an unregulated proliferation of hematopoietic cells, extramedullary hematopoiesis, and fibrosis
Fibroblasts overtake the bone marrow
Found in men and women over 50
Patients have folate deficiency
Moderate leukoerythroblastic anemia that is normochromic, normocytic
Dacrycytes
Elliptocytes
Basophilic Stippling
LDH is normal to increased
Dry Taps
NRBC's
Pseudo-Pelger-Huets
Variable Platelet Counts
Patients live 4-5 years
Term
Polycythemia Vera (PV)
Definition
Clonal Stem Cell Defect
RBC line is the most affected
Seen in people aged 40-60, particularly those of Jewish descent
Hematocrit: >60
30% of patients have thrombosis or hemorrhagic episodes
Plethora
Most patients transition to AML
30% of patients have myelofibrosis
Absolute erythrocytosis with normal to increased retics
Normochromic, normocytic or hypochromic, microcytic anemia
Term
Essential Thrombocythemia
Definition
aka Crimeric Thrombocythemia
aka Hemorrhagic Thrombocythemia
aka Megakaryocytic Leukemia
Affects the platelet line the most
Platelet count: >1-million
Increased EPO
The stem cells have a decreased surface receptor for thrombopoeitin and increased serum levels
Platelets have abnormal functions, and are giant and bizarre
Elevated WBC count
Bone marrow is hyperplastic with increased megakaryocytes
Increased serum-cobalamine, uric acid, LDH, acid phosphatase, and potassium
LAP score is normal to increased
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