Myeloproliferative Syndromes
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- Cronic Myeloproliferative Syndromes
-
-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 - Four major types of chronic myeloproliferative disorders
-
-CML-Chronic myeloid Leukemia
-PV-Polycythemia Vera
-ET-Essential thrombocythemia
-CIMF-Chronic idiopathic myelofibrosis - Two major outcomes of myeloproliferative syndromes
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-Acute Blast Phase
-BM failure due to myelofibrosis - Age of onset of CML
- 5th-6th decade
- Most common myeloproliferative syndrome?
- CML
- Phases of CML
-
-Chronic
-Accelerated
-Blast - Chromosomal abnormality in CML
- Philadelphia chromosome t(9:22)
- Philadelphia chromosome
-
t 9:22 translocation resulting in BCR/ABL gene fusion product
-strongly associated with CML - Symptoms of CML
-
Usually asymptomatic in chronic phase
can have fatigue, weightloss, anemia, night sweats, splenomegaly
symptomswors in accelerated and blast phase - CML and LAP
-
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 - 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 - Characteristics of CML chronic stage
-
-Leukocytosis, basophilia, eosinophilia common
-normal to inc platelets
-anemia
-bm shows myeloid hyperplasio, but blasts <10% - 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 - Characteristics of CML blast phase
-
-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 - Polycythemia Vera
- -Clonal proliferation of RBC's independent of normal regulatory mechanism
- PV age onset
- about 50 years old
- phases of PV
-
Polycythemic phase
Spent Phase - Cytogenic abnormalitis in PV
- NONE
- Transformation in PV?
- Rarely to aml
- Polycythemic Phase of PV
-
-Complications of increased RBC mass
--thrombotic events, ha, dizziness
-ingreased Hgb >18.5 men, 16.5 women
-Thrombocytosis a/o leukocytosis
-splenomegaly - PV diagnosis
-
must exclude secondary causes of erythrocytosis
--hypoxia, smoking, inappropriate EPO secretion by tumor, family history (usually have high EPO)
-Hyperplastic bone marrow - PV Erythropoeitin levels
- Usually low, unlike secondary causes of increased erythropoeisis
- 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) - Essential Thrombocytopenia
-
-Clonal proliferation of megakaryocytes
-Least common Myeloproliferative syndrome - ET age of onset
- 50-60
- ET presentation
- Usually with thrombotic events or paradoxical bleeding episodes
- ET lab values
-
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 - Cytogenic changes in ET
- none
- Other causes of thombocytosis
-
-Reactive-inflamation, infection, splenectomy, iron deficiency, neoplasm, other syndromes
Must exclude these before diagnosising ET - 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 - Symptoms of CIMF
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Vague
-fatigue, dyspnea, weight loss, night sweats, fever, bleeding - CIMF stages
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-prefibrotic
-fibrotic - 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 - 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 - Tear Drop Cells
- RBC's shaped like tear drops that indicate marrow fibrosis (not specific)
- Fibrotic Stage of CIMF
-
-70-80% diagnosed
-Hepatosplenomegaly due to extramedullayr hematopoesis
-Anemia with numerous teardrop cells
LEUKOERYTHROBLASTOSIS (DRAMATIC)
-Variable WBC and Platelet counts - Marrow in Fibrotic stage CIMF
-
-Hypocellular with increased reticulin or collagen fibrosis
-Megakaryocytic hyperplasio
-Osteosclerosis