Myeloproliferative Disorders Flashcards
What are the cell lines affected in Chronic Myeloproliferative Disorders?
CMPD: Cell Line:
ET Megakaryoctic
CML Granulocytic
PV Erythrocytic
IMF Fibroblast/AML
What do we see in the Blast Phase of peripheral blood in Chronic Myelogenous Leukemia?
Blasts greater than 20%
Increase in Promyelocytes
Increase in Baso and Eos
Throbocytopenia
What do we see in the Blast Phase of bone marrow in Chronic Myelogenous Leukemia?
Blasts greater than 20%
Large clusters of blasts
Increased Fibrosis
Marked Dysplasia of all three cell lines
What is the condition that contains an overproduction of RBCs, WBCs, and PLTs that also contains the JAK2 mutation?
Polycythemia Vera (PV)
What is the median survival of Polycythemia Vera (PV)?
more than 10 years
What are synonyms of Primary Myelofibrosis (PMF)?
Myelofibrosis with Myeloid Metaplasia (MMM)
Idiopathic Myelofibrosis (IMF)
Chronic Idiopathic Myelofibrosis
Leukoerythroblastic Anemia
What do we see in a patient that is diagnosed with a form of PMF?
Splenomegaly and Hepatomegaly
50% dry tap due to fibrotic tissue
What is the survival of a patient with PMF?
Median survival 3-5 years
What type of Leukemia do we see 90% - 95% Philadelphia Chromosome in patients?
Chronic Myelogenous Leukemia (CML)
Affecting the entire spectrum of Myeloid Cell development and marked Neutrophil Leukocytosis is diagnostic of which diesease?
Chronic Myelogenous Leukemia (CML)
What can help us differentiate between Chronic Myelogenous Leukemia (CML) and a Leukemoid Reaction?
Leukocyte Alkaline Phosphatase (LAP)
What is diagnostic of Polycythemia Vera (PV) in a CBC?
Pancytosis: Increase in RBCs, WBCs and PLTs
What do we need to distinguish Polycythemia Vera (PV) from?
Secondary Erythrocytosis
Increase in RCM without change in other lines
What do the clinical features of night sweats, weight loss, anorexia, and osteosclerosis most closely resemble?
Primary Myelofibrosis (PMF)
A patient is seen with defective platelets and hemorrhagic and thrombotic episodes. What do we expect to see quantitively with their PLTs?
Increase in PLT production
Hypersensitivity of Mega-K to Cytokines and Thrombopoietin
