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37 Cards in this Set
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Cronic Myeloproliferative Syndromes
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-Clonal stem cell disorders charceterized by proliferation in the bone marrow of
-granulocytes -erythrocytes -megakaryocytes (NOT LYMPHOID) Both BM and PB show proliferation of precursors with normal morphology |
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Four major types of chronic myeloproliferative disorders
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-CML-Chronic myeloid Leukemia
-PV-Polycythemia Vera -ET-Essential thrombocythemia -CIMF-Chronic idiopathic myelofibrosis |
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Two major outcomes of myeloproliferative syndromes
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-Acute Blast Phase
-BM failure due to myelofibrosis |
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Age of onset of CML
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5th-6th decade
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Most common myeloproliferative syndrome?
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CML
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Phases of CML
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-Chronic
-Accelerated -Blast |
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Chromosomal abnormality in CML
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Philadelphia chromosome t(9:22)
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Philadelphia chromosome
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t 9:22 translocation resulting in BCR/ABL gene fusion product
-strongly associated with CML |
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Symptoms of CML
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Usually asymptomatic in chronic phase
can have fatigue, weightloss, anemia, night sweats, splenomegaly symptomswors in accelerated and blast phase |
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CML and LAP
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Leukocyte alkaline phosphatase (LAP) decreased unless concurrent infection
LAP is produced by neutrophils in response to infection, so if there's neutrophilia and no LAP suspect lymphoma |
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CML chronic stage looks like?
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-Leukoerythroblastosis->"bone marrow in the blood"
-immature RBC's inthe blood, neutrophils without reactive toxicity -lots of wbc's |
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Characteristics of CML chronic stage
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-Leukocytosis, basophilia, eosinophilia common
-normal to inc platelets -anemia -bm shows myeloid hyperplasio, but blasts <10% |
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Characteristics of CML accelerated phase
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-Worsening symptoms
-at least ONE of the following --10-19% blasts in PB or BM --20% or more basophils in blood --persistant thrombocytopenia or thrombocytosis --clonal cytogenic evolution |
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Characteristics of CML blast phase
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-Transformtion to AML (or ALL 20% of the time)
--Criteria ->Blasts more than 20% in peripheral blood--KEY ->blast proliferation outsid blood/BM ->Large aggregates of blasts in BM biopsy |
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Polycythemia Vera
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-Clonal proliferation of RBC's independent of normal regulatory mechanism
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PV age onset
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about 50 years old
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phases of PV
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Polycythemic phase
Spent Phase |
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Cytogenic abnormalitis in PV
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NONE
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Transformation in PV?
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Rarely to aml
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Polycythemic Phase of PV
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-Complications of increased RBC mass
--thrombotic events, ha, dizziness -ingreased Hgb >18.5 men, 16.5 women -Thrombocytosis a/o leukocytosis -splenomegaly |
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PV diagnosis
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must exclude secondary causes of erythrocytosis
--hypoxia, smoking, inappropriate EPO secretion by tumor, family history (usually have high EPO) -Hyperplastic bone marrow |
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PV Erythropoeitin levels
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Usually low, unlike secondary causes of increased erythropoeisis
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Spent phase of PV
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-Reticulin fibrosis of BM (stains black)
-Spleen gets bigger (making RBC's) -Leukoerythroblastosis due to extramedullary hematopoeisis (blasts in PB) |
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Essential Thrombocytopenia
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-Clonal proliferation of megakaryocytes
-Least common Myeloproliferative syndrome |
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ET age of onset
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50-60
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ET presentation
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Usually with thrombotic events or paradoxical bleeding episodes
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ET lab values
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Thrombocytosis (pt count >600,000) giant platelets
WBC count my be high, but usually no basophilia -BM shows MEGAKARYOCYTIC HYPERPLASIA with big clusters of megakaryocytes |
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Cytogenic changes in ET
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none
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Other causes of thombocytosis
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-Reactive-inflamation, infection, splenectomy, iron deficiency, neoplasm, other syndromes
Must exclude these before diagnosising ET |
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Chronic Idiopathic myelofibrosis
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-Clonal expansion of granulocytic and megakaryocytic elements with prominent reactive myelofibrosis and extramedullary hematopoeisis
Usually in old people |
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Symptoms of CIMF
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Vague
-fatigue, dyspnea, weight loss, night sweats, fever, bleeding |
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CIMF stages
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-prefibrotic
-fibrotic |
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CIMF signs of poor prognosis
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-Age >70 at diagnosis, Cytogenetic abnormalities, severe anemia, thrombocytopenia, Left shift of granulcytes
-ab 5-30% progress to AML |
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Prefibrotic stage of CIMF
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20-30% pts detected
mild splenomegaly (or none) anemia with TEAR DROP CELLS, throbocytosis, larg atypical platelts, mild or no leukoerythroblastosis |
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Tear Drop Cells
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RBC's shaped like tear drops that indicate marrow fibrosis (not specific)
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Fibrotic Stage of CIMF
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-70-80% diagnosed
-Hepatosplenomegaly due to extramedullayr hematopoesis -Anemia with numerous teardrop cells LEUKOERYTHROBLASTOSIS (DRAMATIC) -Variable WBC and Platelet counts |
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Marrow in Fibrotic stage CIMF
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-Hypocellular with increased reticulin or collagen fibrosis
-Megakaryocytic hyperplasio -Osteosclerosis |