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Myeloproliferative Syndromes


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Cronic Myeloproliferative Syndromes
-Clonal stem cell disorders charceterized by proliferation in the bone marrow of


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
-Acute Blast Phase
-BM failure due to myelofibrosis
Age of onset of CML
5th-6th decade
Most common myeloproliferative syndrome?
Phases of CML
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
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?
-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
-bm shows myeloid hyperplasio, but blasts <10%
Characteristics of CML accelerated phase
-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)

->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
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

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
-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
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
Other causes of thombocytosis
-Reactive-inflamation, infection, splenectomy, iron deficiency, neoplasm, other syndromes

Must exclude these before diagnosising ET
Chronic Idiopathic myelofibrosis
-Clonal expansion of granulocytic and megakaryocytic elements with prominent reactive myelofibrosis and extramedullary hematopoeisis

Usually in old people
Symptoms of CIMF
-fatigue, dyspnea, weight loss, night sweats, fever, bleeding
CIMF stages
CIMF signs of poor prognosis
-Age >70 at diagnosis, Cytogenetic abnormalities, severe anemia, thrombocytopenia, Left shift of granulcytes

-ab 5-30% progress to AML
Prefibrotic stage of CIMF
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
-Variable WBC and Platelet counts
Marrow in Fibrotic stage CIMF
-Hypocellular with increased reticulin or collagen fibrosis

-Megakaryocytic hyperplasio


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