Polycythemia

1. POLYCYTHEMIA
SUNIL KAUMAR.P
Dept.of Haematology
St.John’s Medical College
Bangalore
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2. • Definition
• Classification
• Polycythemia Vera
• Pathogenesis
• clinical features
• laboratory diagnosis
• Rx….
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3. POLY CYTHEMIA
POLY = Many or More
CYTHEMIA = a “ Condition involving cells in
the blood”
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4. DEFINITION
• Polycythemia, or erythrocytosis, refers to an
increase in the number in the RBC mass,
usually with a corresponding increase in
haemoglobin level. The increase in red cells
can be absolute or relative.
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5. Classification
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6. Relative Polycythemia
• Is characterized by decreased plasma volume
with a normal red cell mass.
• They may result from dehydration following
prolonged vomiting, diarrohea or excessive use of
diuretics.
• Gaisbock syndrome ( Spurious polycythemia) is
seen in middle- aged, over weight, hypertensive
and anxious (stressed) individuals, and hence
called as stress polycythemia
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7. Absolute Polycythemia
• Is characterized by a true increase in total red
cell mass and can be sub classified as
• 1. Primary Polycythemia and
• 2. Secondary Polycythemia
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8. • Primary Polycythemia or Polycythemia Vera:
• it results from an intrinsic abnormality of the
hematopoietic stem cell.
• Secondary Polycythemia :
• It results as a compensatory response of red
cell progenitors to an increase in EPO
secretion.
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9. POLYCYTHEMIA VERA
• Polycythemia Vera (PV) is an acquired
myeloproliferative neoplasm arising from
malignant transformation of hematopoietic
stem cell.
• It is associated with mutation of
erythropoietin receptor (EPOR) gene that lead
to autonomous erythropoietin-independent
proliferation of red cell progenitor.
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10. Molecular Pathogenesis in ---- PV
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Signal
Transducers +of
Transcription

11. Pathogenesis in Secondary
Polycythemia
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12. Clinical features
• 1. Age : Usual age of presentation is 50-60 yrs
• 2.Sex: slightly male predominance
• 3.Cynosis
• 4.CNS involvement : Head ache
Loss of memory
Dizziness
Vertigo
Thrombosis – Cerebral vessels
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13. • 5. Visual disturbances
• 6.Skin and mucosal changes
• 7.Splenohepatomegaly
• 8.haemostatic symptoms : Eg : epistaxis
• 9.Budd- Chiari syndrome
• 10.Other C/F :
• Weight loss
Sweating
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14. Laboratory Diagnosis
• PB:
Hb – Increased.
HCT - increased.
RBC - increased and usually about 6 millions /
cumm.
WBC – Normal or increased
PLT – Normal or increase
NAP(LAP)- Score is increased to 150-300
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15. Peripheral blood smear
RBC : Show N/N picture.
WBC’s : TC - increased
There is mild moderate leukocytosis
neutrophils are morphologically Nor.
Basophils often increased
NAP (LAP) score - increased
PLTS: often abnormally large and
functionally defective.
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16. Bone marrow
• Cellularity : hyper cellular
• Hyperplasia of all elements.
• Trilineage hyperplasia of erythroid, myeloid,
megakaryocytic series.
• Prominence of erythroid precursors in the BM.
• Iron stores - NIL
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17. Bone marrow biopsy
• Shows reticulin fibers and fibrosis.
• Extensive marrow fibrosis that displaces
hematopoietic cells.
• Uncommonly, transformation to AML also
occur.
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18. Other Findings
• EPO Levels : Decreased (PV)
: Increased in (secondary PV)
• Serum Vit.B12: Increased.
• JAK2 V617F or other functionally similar
mutation can be demonstrated
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19. Cause of Death
• Most of the patients die of vascular
complications.
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20. Treatment
• Venesection
• Myelosupression
• Alpha-Interferon
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