Qualitative Wbc Disorders

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Qualitative WBC disorders

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• Observe cells on stained blood smear
• Inherited: insignificant or life threatening
• Acquired: indicate disease state
• Types of changes: cytoplasmic (more common) or nuclear

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Acquired: Cytoplasmic changes

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• Cytoplasmic inclusions found in infectious states
• Toxic granulation
• Toxin vacuolization
• Dohle bodies
• Intracellular organisms

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Toxic granulation

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• Acquired Cytoplasmic change
• heavy coarse granules evenly distributed in the cytoplasm

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Toxic vacuolization

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• Acquired cytoplasmic changes
• Clear areas of varying sizes representing rupture of granules

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Dohle bodies

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• Acquired cytoplasmic changes
• pale blue cytoplasmic inclusions in neutrophils and eosinophils near periphery of cell
• Composed of aggregates of rough endoplasmic reticulum

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Intracellular organism

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• Acquired cytoplasmic changes
• Bacteria/fungi

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Function of normal granulocytes

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• Neutrophils
• Chemotaxis
• Phagocytosis
• degranulation
• intracellular microbial killing

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Toxic granulation

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Indicates cells death

Chemotherapy

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aggregrates of rough ER

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Inherited WBC disorders cytoplasmic changes

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• Chediak Higashi syndrome
• May-Hegglin anomaly
• Alder-Reilly anomaly
• Chronic granulomatous disease
• Other neutrophil dysfunction
• Suspected with recurring infections, no response to antibiotics, delayed wound healing, resisitant sepsis

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Chediak Higashi Syndrome

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• Autosomal recessive
• Giant cytoplasmic granules in neutrophils, lymphocytes, and monocytes
• Albinism, photophobia, neuropathy
• Reduced chemotaxis and bacterial killing
• Recurrent bacterial infection

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Chediak-Higashi Syndrome

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May-Hegglin Anomaly

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• Thrombocytopenia
• Giant Platelets
• Dohle-like bodies present in neutrophils, eosinophils, basophils, monocytes, and in giant platelets

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May-Hegglin Anomaly

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Alder-Reilly Anomaly

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• Def. of enzymes for the breakdown of mucopolysacchrides
• Large, dark, purplish granuls in the cytoplasm of all leukocytes contain mucopolysacchrides in disorders like Hurler's and Hunter's syndrome
• Complication: deposition of mucopolysacchrides in the tissues

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Alder-Reilly anomaly

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CGD

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• Inherited disorder defects in respiratory burst oxidase system
• Cannot generate antimicrobal oxygen metabolites
• recurrent infections with opportunistic infections causing granulomas
• Neutrophil morphology is normal
• Ineffective bacterial killing

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Nuclear changes

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• Hypersegmentation (more than 5)
• Hyposegmentation:
• Inherited: Pelger-Huet anomaly
• Acquired: pseudo-pelger-huet

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Twinning deformity

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Pelger-Huet anomaly

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• Hyposegmentation
• Dominant
• Homozygous-90% of neutrophils have round single nucleus in 98% of cases
• Heterozygous- neutrophils with bi-segmented nucleus in 98% of cases, many band neutrophils with pyknotic infection

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Pelger-Huet anomaly

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Infectious Mononucleosis

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• Acute, febrile, self-limiting viral infection
• EBV
• Lymphocytes effected
• High in adolescents
• Soar throat, fatigue, cervical node adenopathy
• less than 50% splenomegaly
• 25% hepathomegaly

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Laboratory Tests IM

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• Lymphocytes less then 50%
• greater than 10% reactive lymphs
• Serology: monospot test, specific viral test
• Liver function tests

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Reactive

Variant Lymphocytes

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Mononucleosis Syndrome

Cytomegalovirus Infection

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• Viral infection; caused by CMV
• Hematologic changes: same as IM
• Heterophile antibody (Monospot test): negative

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Other causes for reactive lymphocytes

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• Viral hepatitis
• Drug hypersensitivity
• Toxoplasmosis
• Other viral infections

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Sphingolipid metabolism

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• enzyme defects
• sphingomyelin accumulates in Niemman Pick defect in sphingomyelinase
• Gaucher: defect in Beta-glucosidase; can't breakdown glycosylceramide

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Gaucher cells (monocyte/histocytes)

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Niemann-Pick cell