Neutropenia, Agranulocytosis
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Neutropenia, Agranulocytosis

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Neutropenia, Agranulocytosis Presentation Transcript

  • 1. LEUKOPENIA
    Abnormally LOW WBC
  • 2. NEUTROPENIA, AGRANULOCYTOSIS
  • 3. NEUTROPENIA
    Reduction in the number of granulocytes in PBS
    (peripheral blood smear)
    < 2000/uL
    Mild 1000-2000/uL
    Moderate 500-1000/uL
    Severe (agranulocytosis) <500/uL
    DEFINITION:
  • 4. AGRANULOCYTOSIS
    Marked reduction in neutrophil count
    Disappearance of neutrophil precursors in the bone marrow
    Absence or less than 500/uL of pmns
  • 5. PATHOGENESIS:
    1. INEFFECTIVE GRANULOPOIESIS
    2. ACCELERATED REMOVAL OR DESTRUCTION OF NEUTROPHILS
  • 6. Multipotent Progenitors
    Commited Precursors
    Late
    Precurosors
    Mature
    Form
  • 7. INEFFECTIVE GRANULOPOIESIS
    Suppression of hematopoietic stem cells
    Aplastic anemia
    Infiltrative d/o
    Suppression of committed granulocytic precursors
    Alkylating drugs – generalized effect
    Idiosyncratic reaction – Chloramphenicol
  • 8. Defective precursor cells susceptible to death while in BM
    Megaloblastic anemia
    Genetic defect  impaired granulocytic differentiation : Kostmann syndrome
    INEFFECTIVE GRANULOPOIESIS
  • 9. Immune mediated
    Idiopathic
    Autoimmune
    Drugs
    Splenic sequestration
    20 to enlargement of spleen
    Increased peripheral utilization
    Overwhelming infection
    ACCELERATED REMOVAL/DESTRUCTION
  • 10. Most common cause is DRUG TOXICITY
    Alkylating drugs/ Anti-metabolites
    Generalized suppresion of BM  Agranulocytosis
    Predictable
    Dose related
    Chlorpromazine/ Phenothiaxines
    Toxinc effect on granulocytic precursors in BM
    Thiouracil/Sulfonamides/ Aminopyrine
    Antobody mediated destruction of mature neutrophils
    Autoantibodies against neutrophil specific antigens
    Suppression of marrow granulocytic progenitors by products of neoplastic cell
    Agranulocytosis:
  • 11. S/S Related to infection
    Malaise, chills, fever
    Marked weakness & fatigue
    Death in severe agranulocytosis
    Neutrophil count < 500/mm3
    CLINICAL COURSE:
  • 12. Broad spectrum antibiotic
    G-CSF , a growth factor that stimulates production of granulocytes from BM
    Given following chemotx
    Withdrawing or tapering dose of drug
    Treatment:
  • 13. LEUKOCYTOSIS
    INCREASE IN NUMBER OF WBC
    • REACTIVE
    • 14. FIRST SIGN OF NEOPLASTIC GROWTH OF WBC
  • Pathogenesis
  • 15.
  • 16.
  • 17.
  • 18. CAUSES:
  • 19. CAUSES:
  • 20. ACUTE NONSPECIFIC LYMPHADENITIS
  • 21. Reactive changes to:
    Microbe , Cell debris, Foreign matter
    Localized – Regional LN
    Generalized Lymphadenopathy
    Systemic Viral infection – Children
    Bacteremia
    Prominence of Lymphoid Follicles with Large germinal center, Debris, Macrophages, Necrosis, Suppuration
    Features:
  • 22. Enlarged
    Tender to touch
    Fluctuant if (+) abscess
    Draining sinuses to skin
    Suppurative necrosis
    Clincal Presentation:
  • 23. CHRONIC NONSPECIFIC LYMPHADENITIS
    MORPHOLOGIC TYPES
  • 24. Activation of Humoral response
    Rheumatoid arthritis
    Early stage of HIV
    Must be differentiated from Follicular Lymphoma
    Germinal center B-cells are (+) for BCL2 stain
    FOLLICULAR HYPERPLASIA
  • 25. Morphology
  • 26. Trigger the Cellular Immune responses
    Reactive changes in T cell region of LN
    PARACORTICAL AREA EXPANSION
    EFFACED THR FOLLICLES
    Drugs – Dilantin
    Acute viral infxn
    IM
    Viral Vaccine
    PARACORTICAL LYMPHOID HYPERPLASIA
  • 27. PARACORTICAL LYMPHOID HYPERPLASIA
  • 28. Distention of lymphatic sinusoids
    Non-specific
    Particularly prominent in LN draining cancers
    Represent host immune reaction to cancer
    Sinus Histiocytosis
  • 29. Sinus Histiocytosis