O. Myeloproliferative Disorders Flashcards
What are the 4 types of Myeloproliferative disorder?
CML
Polycythemia Vera
Essential Thrombocytopenia
Myelofibrosis
What is the Myeloproliferative disorder of the RBCs?
PV
What is the Myeloproliferative Disorder of platelets?
ET
What is the myeloproliferative disorder of granulocytes?
CML
What age group do the myeloproliferative disorders generally appear in? Exception?
Middle aged or elderly
CML can sometimes present in children
Chronic Myeloproliferative Disorders can progress to what?
Acute Leukemia
What cytogenic abnormality is associated with CML? What is the test for it?
t(9:22)
ABL on 9 & BCR on 22
Philadelphia Chromosome = fusion –> tyrosine kinase activity
FISH
Clinical presentation of CML?
Some are asymptomatic
Fatigue, weakness, weightloss
Striking leukocytosis, granulocytic hyperplasia with left shift, basophils present
What is a LAP (Leukocyte Alkaline Phosphatase) used for?
To differentiate between CML & reaction to infection
Low/ansent in CML
High in infection
What are the 3 stages of CML? Survival for each? % Blasts for each?
Chronic = 20% blasts = acute leukemia
The blast crisis stage of CML looks exactly like what?
AML
Which stage of CML is unresponsive to treatment?
Accelerating phase
What is the treatment for CML?
Gleevec/imatinib = tyrosine kinase inhibitor
CML is a build up of what?
Pluripotent stem cell that could become myeloid or lymphoid (but mostly becomes myeloid)
What physical finding is common with CML?
Splenomegaly
What 2 neoplastic disorders have the philedelphia chromosome?
CML
ALL
What are the clinical findings for PV?
Elevated Hct Headache HTN Itchy skin Splenomegaly Thrombosis and bleeding Elevated WBC Elevated platelets
What is suggested with clusters of megakaryocytes?
PV
What is the treatment for PV?
Phlebotomy or hydroxyurea
Differential diagnosis for PV?
Hypoxemia
Smoking
High affinity hemoglobin
High EPO
PV is an overproduction of what?
Erthrocytes, granulocytes & megakaryocytes
What are the 3 stages of PV and the survival for each?
Chronic = 10-15 years
Spent Phase = 2-3 years
Acute Leukemia =
What is the clinical presentation of Essential Thombocythemia?
Thrombocytosis
Some asymptomatic
Bleeding and thrombosis
What is suspected with aggregates of platelets?
ET
How does the bone marrow look in ET?
Could be hypercellular or normal
Increased megakaryocytes
What is Primary Myelofibrosis?
Proliferation of granulocytes, megakaryocytes associated with bone marrow fibrosis (connective tissue) & extramedullary hematopoesis
What are the 2 stages of myleofibrosis?
Prefibrotic = proliferative stage. Mild hepatosplenomegaly, mild blood changes Fibrotic = symptoms
What does a positive stain for reticulin suggest?
Myelofibrosis
What do tear drop shaped RBCs suggest?
Myelofibrosis
What does excessive pink staining on a bone marrow biopsy suggest?
Myelofibrosis
What is leukoerythroblastic appearance on a smear?
Nucleated erythroid cells, left shifted myeloid cells, darcocytes
What mutation is seen in all of the myeloproliferative disorders accept CML?
Jak2 muation –> constituitively active tyrosine kinase
How do the 4 myeloproliferative disorders present initially?
CML‐high WBC, PLT
PV‐high RBC/ Hgb, WBC, PLT
ET‐typically only high PLT
CIMF‐high WBC, PLT
How do the 4 myeloproliferative disorders present after a prolonged course?
All CMPD may progress (rarely with ET, invariably with CIMF) to marrow fibrosis and morphologically resemble CIMF, at this stage one cannot reliably determine what it was before; now the peripheral blood counts may be low/ normal
What is a myelodysplastic disorder?
Clonal defect in stem cells which lead to defects in cell differentiation & ineffective erythropoesis
Key difference between myelodysplastic and myeloproliferative disorders?
myelodysplastic = decreased production of cells Myeloproliferative = increase in production of cells
What are the 2 main types of myelodysplastic syndromes?
Primary = unknown cause, most related to cytogenic abnormalities
Therapy Related = 2-8 years after treatment with radiation or cytotoxic (akylating gents or topoisomerase)
What are the clinical presentations of MDS?
Anemia, nuetropenia & thrombocytopenia (+ associated symptoms)
Low retic count
Macrocytic RBCs (High MCV)
Hypercellular marrow
What is suggested by psuedo pelger huet cells? What do they look like?
MDS
Bilobed nuclei of nuetrophil = sun glasses
Ringed sinderoblasts can be seen with what neoplastic disorder?
MDS
Basophils are characteristically elevated in what?
CML
What is a common secondary cause of polycythemia?
smoking
What system determines the prognosis of MDS?
international prognostic scoring system (IPSS)
