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22 Cards in this Set
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MPN
Def. Notes |
Disorders of the hemaotopoietic stem cell that result in over production of erythrocytes, granulocytes, and/or platelets
They result in the over accumulation of these cells in the marrow and/or blood |
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MPN
Characteristics |
Location of hematopoiesis
Production of cells
Blast cells
Transformation |
Extramedullary hematopoiesis Liver spleen
Panmyelosis in all cell lines
Less than 20% blast cells
Malignant transformation to acute leukemia |
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Cell lines
PV |
Erythroid |
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Cell lines
CML, CNL, CEL, Mastocytosis |
Myeloids |
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Cell lines
ET |
Megaryocytic |
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Cell lines
PMF |
Fibroblast |
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CML
full name |
Chronic myelocytic leukemia |
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CML
Notes
Population Def. Progression |
Disease of middle age (46-53), male and female
Affect young adults more than the other MPNs
Neoplastic growth of primarily the myeloid cells
Extramedullary granulocytic proliferation in spleen and liver
Disease is progressive |
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CML
Phases of this disease |
Three phases
What are they? |
Chronic Accelerated Acute (Blastic phase) |
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CML
Cause |
Philadelphia chromosome
Notes |
Philadelphia chromosome t(9,22) in 95% of patients (better prognosis)
Express enhanced tryosine kinase activity
Drives cell proliferation Overrides normal regulatory control (apoptosis) |
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CML
Symptoms/clinical signs |
Anemia |
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CML
Blood findings |
Extreme leukocytosis (200-500x10^9)
Platelets increased in over half of patients early in disease
Shift to the left with a myeloctyic peak
Blast count: exceed 10%
+ Eos and bas
Low LAP |
WBC Platelets Blast count Eos and Bas LAP |
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CML
Blood findings
WBC |
Extreme leukocytosis
Data |
200-500x10^9 |
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CML
Blood findings: Platelets |
+ in 50% of patients early in disease |
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CML blood findings
Maturity |
Shift to the left with a myelocytic peak |
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CML
Blood findings Blast count |
Exceed 10% |
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CML
Eosinophils and basophils |
+ |
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CML
LAP |
Low |
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CML
Three phases |
Chronic Accelerated Blast |
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CML
Chronic phase
Notes |
Onset Symptoms Period |
Insidious onset Symptoms are often not present Increased weakness, unexplained fever, night sweats and weight loss
The chronic phase may last several months |
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CML
Blast phase Notes |
Number of blast Symptoms Survival |
There are 20% or more blast cells in the blood/bone marrow. Symptoms worsen Survival is measured in months |
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CML vs. infection |
Blast and promyelocytes
Toxic changes Dohle bodies Basophilia and eosinophilia |
More mature vs. rare
Absent vs. present (Toxic granules and cytoplasmic vaculization)
No dohle bodies vs. Dohle bodies
+ vs. - |