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18 Cards in this Set

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What are the characteristics of Chronic Myeloproliferative Diseases?
1) peripheral blood cytosis (leukocytosis, thrombocytosis)
2) anemia
3) splenomegaly, hepatomegaly
4) bone marrow hypercellularity
What is the definition of Chronic Myelogenous Leukemia?
It is a myeloproliferative disorder from a pluripotent bone marrow stem cell, with Philadelphia chromosome or BCR-ABL fusion gene.
What is the epidemiology of CML?
1) 15-20% of all leukemias
2) older patients, 50's and 60's
What is the Philadelphia chromosome?
1) short chromosome 22
2) t(9;22)
3) contains BCR-ABL fusion
4) major breakpoint p210 in CML
What does the BCR-ABL fusion gene do?
1) has increased tyrosine kinase activity
2) increases transcription of MYC and BCL-2, which protects leukemic cells from apoptosis
3) targeted therapy: Gleevec
What do you see in the Chronic Phase (1st) of CML?
1) peripheral blood shows leukocytosis - mainly PMNs
2) bone marrow is hypercellular with small hypolobated megakaryocytes
what do you see in the Accelerated Phase (2nd) of CML?
1) basophilia > 20%
2) marked bone marrow fibrosis
What do you see in the Blast Phase (3rd; transformation to AML) of CML?
1) basophilia > 20%
2) extramedullary blast proliferation
3) bone marrow biopsy showing large foci or clusters of blasts
What is Polycythemia Vera?
1) myeloproliferative disease arising from hematopoietic stem cell
2) increased RBC production independent of EPO
How do you diagnose PV?
* 1) increased Hb or RBC mass
* 2) no secondary causes of erythrocytosis
3) no Philadelphia chromosome or BCR-ABL
4) thrombocytosis > 400,000
5) WBC > 12,000
6) low serum EPO
* 7) 75% of patients have JAK2 mutation
What do you see in the Proliferative Phase of PV?
1) predominantly erythroid proliferation in bone marrow
2) hypercellular bone marrow
3) lack stainable iron
What do you see in the Spent Phase of PV?
1) fibrotic marrow with collagen
2) teardrop shaped RBCs
What is Chronic Idiopathic Myelofibrosis?
myeloproliferative disorder, mainly of megakaryocytes
What are the 2 phases of CIMF?
1) pre-fibrotic: mild
2) fibrotic: marked hepatosplenomegaly + marked anemia + teardrop RBCs
What is Essential Thrombocythemia?
myeloproliferative disorder, mainly of megakaryocytes (high PL count for over 6 months)
What do you have to rule out in order to diagnose ET?
1) CML, PV, CIMF
2) myelodysplastic syndrome
3) infection, inflammation, neoplasm, iron deficiency

diagnosis of exclusion!!!
What is RARS?
1) myelodysplastic disease, refractory anemia with ringed sideroblasts
2) erythroid precursor nucleus surrounded by blue iron granules
3) best prognosis of the MDS
What are characteristics of Myelodysplastic Disease?
1) ineffective hematopoiesis, leading to anemia, thrombocytopenia, bone marrow hypercellularity
2) dysplastic precursors
3) idiopathic, or therapy-related etiology