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8/14/2019 Other Leukocyte Disorders
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Other LeukocyteOther Leukocyte
DisordersDisorders
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Hema-20Hema-20 33
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Hema-20Hema-20 44
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Hema-20Hema-20 55
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Hema-20Hema-20 66
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Hema-20Hema-20 88
Myeloproliferative DisordersMyeloproliferative Disorders
Acute FAB M0 to M7Acute FAB M0 to M7 Chronic distinguished byChronic distinguished by
predominate cell typepredominate cell type Affects middle age & olderAffects middle age & older Insidious onsetInsidious onset BM fibrosisBM fibrosis TransitionsTransitions Platelet abonormalitiesPlatelet abonormalities
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Hema-20Hema-20 99
Chronic MyelogenousChronic Myelogenous
LeukemiaLeukemia
CML with all its variationsCML with all its variations
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Hema-20Hema-20 1010
Polycythemia veraPolycythemia vera
ErythrocytosisErythrocytosis
Primary causePrimary cause
Secondary causesSecondary causesNormal response to abnormal eventNormal response to abnormal event
AltitudeAltitude
Increases activityIncreases activityPregnancyPregnancy
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Hema-20Hema-20 1111
Polycythemia veraPolycythemia vera
Excessive proliferation ofExcessive proliferation of
myeloid & megakaryocyticmyeloid & megakaryocytic
elements in BM & Bloodelements in BM & Blood
Absolute increase in RBC massAbsolute increase in RBC mass
Abnormal pluripotent stem cellsAbnormal pluripotent stem cells
>50 yo, avg 60 yo>50 yo, avg 60 yo Ashkenazi Jews 10:1 ArabsAshkenazi Jews 10:1 Arabs
Etiology UnknownEtiology Unknown
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Hema-20Hema-20 1212
Polycythemia veraPolycythemia vera
Clinical featuresClinical featuresProgressive increase in Hct, Hgb,Progressive increase in Hct, Hgb,
RBCRBC
Headaches, dizziness, visionHeadaches, dizziness, vision
problemsproblems
Bleeding problemsBleeding problems
SplenomegalySplenomegalyRuddy ComplexionRuddy Complexion
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Hema-20Hema-20 1313
Polycythemia veraPolycythemia vera
Laboratory FindingsLaboratory Findings Increase RBC, Hct, HbgIncrease RBC, Hct, Hbg
May develop Iron deficiency,May develop Iron deficiency,
MicrocytosisMicrocytosis
Reticulocytes normalReticulocytes normal
Platelets increasePlatelets increase
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Hema-20Hema-20 1414
Polycythemia veraPolycythemia vera
TreatmentTreatmentPhlebotomyPhlebotomy
Tried P*, not goodTried P*, not good
Prognosis 8 15 yearsPrognosis 8 15 yearsButBut
BM wears ooutBM wears oout
Conversion to Acute formsConversion to Acute forms
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Hema-20Hema-20
1616
Essential ThrombocythemiaEssential Thrombocythemia
TreatmentTreatmentAspirinAspirin
ChemoChemo
InterferonInterferon
Prognosis > 5 yearsPrognosis > 5 years
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Hema-20Hema-20
1717
Myelofibrosis with MyeloidMyelofibrosis with Myeloid
MetaplasiaMetaplasia
Fibrosis of Bone MarrowFibrosis of Bone Marrow
Multiple possible symptomsMultiple possible symptoms
Age 50 70 yoAge 50 70 yo Male 2:1Male 2:1
Less common in AfricansLess common in Africans
Etiology unknownEtiology unknown
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Hema-20Hema-20 1818
MyelofibrosisMyelofibrosis
Decrease RBCDecrease RBC Increase PLTIncrease PLT Fibrosis in BM, Dry tapFibrosis in BM, Dry tap Splenomegaly, LiverSplenomegaly, Liver Anemia becomes progressiveAnemia becomes progressive Bizarre RBC morphologyBizarre RBC morphology Treat symptomsTreat symptoms Prognosis varies months to > 10yPrognosis varies months to > 10y
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Hema-20Hema-20 1919
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Hema-20Hema-20 2020
Myelodysplastic syndromesMyelodysplastic syndromes
Refractory Anemia (RA)Refractory Anemia (RA)
RA with ringed sideroblastsRA with ringed sideroblasts
RA with excess blasts (RAEB)RA with excess blasts (RAEB) RAEB in transformationRAEB in transformation
Chronic MyelomonocyticChronic Myelomonocytic
LeukemiaLeukemia
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Hema-20Hema-20 2121
Myelodysplastic syndromesMyelodysplastic syndromes
Elderly, ~ 70 yoElderly, ~ 70 yo
Few youngerFew younger
Etiology unknown, butEtiology unknown, but Toxins maybe linkedToxins maybe linked
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Hema-20Hema-20 2222
Refractory Anemia (RA)Refractory Anemia (RA)
Difficult to Recognize &Difficult to Recognize &diagnosisdiagnosis
FAB categoriesFAB categories Based on Qualitative differencesBased on Qualitative differences RARA
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Hema-20Hema-20 2323
RA with ringed sideroblastsRA with ringed sideroblasts
RARA15% ringed sideroblasts
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Hema-20Hema-20 2424
RA with excess blasts (RAEB)RA with excess blasts (RAEB)
RARA
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Hema-20Hema-20 2525
RAEB in transformationRAEB in transformation
RARA>5% Blasts>5% Blasts
CytopeniaCytopenia
3 different cell lines3 different cell lines
BM 20 - 30% blastsBM 20 - 30% blasts
5% 20% Auer Rods5% 20% Auer Rods
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Hema-20Hema-20 2626
Chronic MyelomonocyticChronic Myelomonocytic
LeukemiaLeukemia
RARA20% Monocytic
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