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18 Cards in this Set
- Front
- Back
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What are the characteristics of Chronic Myeloproliferative Diseases?
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1) peripheral blood cytosis (leukocytosis, thrombocytosis)
2) anemia 3) splenomegaly, hepatomegaly 4) bone marrow hypercellularity |
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What is the definition of Chronic Myelogenous Leukemia?
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It is a myeloproliferative disorder from a pluripotent bone marrow stem cell, with Philadelphia chromosome or BCR-ABL fusion gene.
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What is the epidemiology of CML?
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1) 15-20% of all leukemias
2) older patients, 50's and 60's |
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What is the Philadelphia chromosome?
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1) short chromosome 22
2) t(9;22) 3) contains BCR-ABL fusion 4) major breakpoint p210 in CML |
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What does the BCR-ABL fusion gene do?
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1) has increased tyrosine kinase activity
2) increases transcription of MYC and BCL-2, which protects leukemic cells from apoptosis 3) targeted therapy: Gleevec |
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What do you see in the Chronic Phase (1st) of CML?
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1) peripheral blood shows leukocytosis - mainly PMNs
2) bone marrow is hypercellular with small hypolobated megakaryocytes |
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what do you see in the Accelerated Phase (2nd) of CML?
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1) basophilia > 20%
2) marked bone marrow fibrosis |
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What do you see in the Blast Phase (3rd; transformation to AML) of CML?
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1) basophilia > 20%
2) extramedullary blast proliferation 3) bone marrow biopsy showing large foci or clusters of blasts |
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What is Polycythemia Vera?
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1) myeloproliferative disease arising from hematopoietic stem cell
2) increased RBC production independent of EPO |
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How do you diagnose PV?
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* 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 |
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What do you see in the Proliferative Phase of PV?
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1) predominantly erythroid proliferation in bone marrow
2) hypercellular bone marrow 3) lack stainable iron |
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What do you see in the Spent Phase of PV?
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1) fibrotic marrow with collagen
2) teardrop shaped RBCs |
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What is Chronic Idiopathic Myelofibrosis?
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myeloproliferative disorder, mainly of megakaryocytes
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What are the 2 phases of CIMF?
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1) pre-fibrotic: mild
2) fibrotic: marked hepatosplenomegaly + marked anemia + teardrop RBCs |
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What is Essential Thrombocythemia?
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myeloproliferative disorder, mainly of megakaryocytes (high PL count for over 6 months)
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What do you have to rule out in order to diagnose ET?
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1) CML, PV, CIMF
2) myelodysplastic syndrome 3) infection, inflammation, neoplasm, iron deficiency diagnosis of exclusion!!! |
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What is RARS?
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1) myelodysplastic disease, refractory anemia with ringed sideroblasts
2) erythroid precursor nucleus surrounded by blue iron granules 3) best prognosis of the MDS |
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What are characteristics of Myelodysplastic Disease?
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1) ineffective hematopoiesis, leading to anemia, thrombocytopenia, bone marrow hypercellularity
2) dysplastic precursors 3) idiopathic, or therapy-related etiology |
