4. AGRANULOCYTOSIS
• The term "agranulocytosis" (Shultz syndrome)
is used when neutropenia occurs as a reaction
(most probably immunologic) to drugs.
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5. DEFINITION
• Neutropenia/ agranulocytosis is the term
when neutrophil count is <2.5 x 109/L and
children and < 1.5 x 109/L.
Mild -when the count is 1000-1800/mm3
Moderate- when the count is 500-
1000/mm3
Severe -When the ANC isbelow 500/mm3
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6. EPIDEMIOLOGY
• Frequency :
• The exact frequency of agranulocytosis is
unknown.
• The estimated frequency of agranulocytosis is
1.0 -3.4 cases per million population per year.
• Race :
• Agranulocytosis has no racial predilection
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7. • Sex:
• Agranulocytosis occurs slightly more frequent
in women than in men, possibly because of
their increased rate of medication usage.
• Whether this higher frequency is related to
the increased incidence of autoimmune
disease in women is unknown.
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8. • Age :
• Agranulocytosis occurs in all age groups.
• The congenital forms are most common in
childhood.
• Acquired agranulocytosis is most common in
the elderly population
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13. PATHOGENESIS
• Pathogenesis of neutropenia can be divided
into following categories
• 1. Inadequate or ineffective formation of
granulocytes.
• 2.Accelerated destruction of neutrophils
• 3.Decreased production
• 4.Decreased survival
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14. 1. Inadequate or ineffective formation
of granulocytes.
• This can be due to bonemarrow failure such
that occurs in aplastic anaemia, several
leukaemias and chemotherapeutic agents.
• There can also be isolated neutropenias where
only differentiated granulocyte precursors are
affected as in the case of neoplastic
proliferation of cytotoxic T cells or NK Cells
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15. 2.Accelerated destruction of
neutrophils
• Immune mediated reactions to neutrophils
which can be caused by drugs.
• An enlarged spleen can lead to splenic
sequestration and accelerated removal of
neutrophils.
• Utilization of neutrophils can occur in
infection.
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19. CLINICALFEATURESOFNEUTROPENIA
• The manifestations of neutropenia depend
upon the degree of neutropenia and of
compensatory monocytosis.
• The manifestations are generally due to severe
bacterial infections.
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24. Peripheral Blood
• Complete blood examination is required to
rule out various causes of pancytopenia
• Hb : Normal
• TLC: Decreased
• DLC: Neutropenia with neutrophils being
0-20% .
• ANC: < 0.5 X 109/L
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25. Bone marrow Examination
• BM is essential to rule other causes of
neutropenia like…….
• sub leukemic/
• aleukemic leukemia
• Megaloblastic anemia
• Aplastic anemia
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26. • Cellularity : Normal
• Erythropoiesis: Normoblastic
• Myelopoiesis: Myelopoiesis in No. of
cases manifests maturation arrest at
promyelocytes / myelocyte stage.
• In some cases there is depletion of the cells of
myeloid series.
• Megakaryopoiesis : Normal
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