Myeloproliferative Disorders/Neoplasms (MPDs/MPNs) Questions & Answers
1. Common find- 1. Overlapping lab results
ings in MPDs 2. Increase in 1 or more cell lines
3. Chronic onset in middle age adults
4. Pronounced splenomegaly
5. Ends in acute leukemia
2. MPNs according 1. Polycythemia Vera Rubra (PV)
to FAB 2. Chronic Myelogenous Leukemia (CML)
3. Essentially Thrombocythemia (ET)
4. Chronic Idiopathic Myelofibrosis (CIMF)
3. polycythemia True malignancy -- has to be ditterentiated between relative polycythemia and
vera rubra (PV) vs secondary polycythemia
other forms of
polycythemia Relative Polycythemia
Not a true increase in cell volume, but rather caused by a decrease in plasma
volume
Seen in dehydrated patients
Has Normal EPO levels and normal CBC findings in most cases
Secondary Polycythemia
True increase in cell volume, but due a secondary factor attecting oxygen demand
Secondary factors include:
1. High altitudes
2. Smoking cigarettes
3. High blood pressure
1/5
, Myeloproliferative Disorders/Neoplasms (MPDs/MPNs) Questions & Answers
Has increased EPO levels and increased RBC findings
Polycythemia Vera Rubra (PV)
True malignancy caused by mutation in PSC independent of EPO levels
EPO levels = can be normal/elevated/decreased
4. Polycythemia Produces a Panmyelosis = causes increase in all 3 cell lines
Vera Rubra (PV) Most prominent in the RBC line, but attects all 3.
Caused by a JAK2 mutation occurring in the Pluripotential Stem Cell (PSC)
Bone marrow findings:
Increased Normoblasts
Increased Granulocyte Precursors
Increased Megakaryocytes
Increased Reticulin
Peripheral Blood findings:
Increased RBC, H&H
Increased WBC count
Increased PLTs
Cytochemical stains: Not used unless transforming into AML
Flow cytometry: not helpful because attects all cell lines
Cytogenetics/Molecular: JAK2 mutation (~90% positive)
2/5
1. Common find- 1. Overlapping lab results
ings in MPDs 2. Increase in 1 or more cell lines
3. Chronic onset in middle age adults
4. Pronounced splenomegaly
5. Ends in acute leukemia
2. MPNs according 1. Polycythemia Vera Rubra (PV)
to FAB 2. Chronic Myelogenous Leukemia (CML)
3. Essentially Thrombocythemia (ET)
4. Chronic Idiopathic Myelofibrosis (CIMF)
3. polycythemia True malignancy -- has to be ditterentiated between relative polycythemia and
vera rubra (PV) vs secondary polycythemia
other forms of
polycythemia Relative Polycythemia
Not a true increase in cell volume, but rather caused by a decrease in plasma
volume
Seen in dehydrated patients
Has Normal EPO levels and normal CBC findings in most cases
Secondary Polycythemia
True increase in cell volume, but due a secondary factor attecting oxygen demand
Secondary factors include:
1. High altitudes
2. Smoking cigarettes
3. High blood pressure
1/5
, Myeloproliferative Disorders/Neoplasms (MPDs/MPNs) Questions & Answers
Has increased EPO levels and increased RBC findings
Polycythemia Vera Rubra (PV)
True malignancy caused by mutation in PSC independent of EPO levels
EPO levels = can be normal/elevated/decreased
4. Polycythemia Produces a Panmyelosis = causes increase in all 3 cell lines
Vera Rubra (PV) Most prominent in the RBC line, but attects all 3.
Caused by a JAK2 mutation occurring in the Pluripotential Stem Cell (PSC)
Bone marrow findings:
Increased Normoblasts
Increased Granulocyte Precursors
Increased Megakaryocytes
Increased Reticulin
Peripheral Blood findings:
Increased RBC, H&H
Increased WBC count
Increased PLTs
Cytochemical stains: Not used unless transforming into AML
Flow cytometry: not helpful because attects all cell lines
Cytogenetics/Molecular: JAK2 mutation (~90% positive)
2/5
