Leukocyte Qualitative Disorders

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Leukocyte Qualitative Disorders

• Presented byDr Akram Saadeh
• ModeratorDr Yousef Abu Osba

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Neutrophil rolling
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Evaluation for phagocytic cell disorder

1. Tow or more systemic bacterial inf.
2. Three or more serious respiratory or documented
   bacterial inf.
3. Inf. Occurring at unusual sites.
4. Inf. with unusual pathogens.
5. Inf. With common childhood pathogens but of
   unusual severity.

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Disorders of neutrophil function
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Job syndrome

• Disorder of chemotaxis.
• AD.
• Increase IgE LEVEL.
• Recurrent Staph. Abscesses(cold abscesses).
• Course features.
• Osteoporosis.
• Recurrent pneumonias.esophagitismeningitis

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Job synd. Atopic dermatitis

• Increase IgE Increase IgE
• Severe dermatitis Severe dermatitis.
• Well defined Well defined
  
• erythematous erythematous
• maculopapular rash maculopapular rash.
• 1st few days-weeks Not in the 1st few mo
• Extensor surfaces Flexor surfaces.

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Job syndrome
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Job syndrome
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Job syndrome
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Job syndrome
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Job syndrome-DxMx

• High IgE, mild increase in IgD.
• NR WBC,eosinophils up to 50.
• Soft tissue syst. Inf. broad spectrum AB.
• Fungal inf.
• Gamma IF.
• Prophylaxis.

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LAD

• AR.
• Decreased or absent expression of a family of
  structurally functionally related leucocyte
  surface glycoprotein designated the CD11/CD18
  complex leading to failure of adherance.
• Recurrent soft tissue inf.
• Delayed wound healing.
• Severely impaired pus formation.
• Delayed separation of the umbilical cord.

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LAD-DxMx

• Flow cytometry measurement of surface CD11/CD18.
• Supportive.
• Prophylaxis.
• BMT.
• Prognosisoften not surviving beyond
  toddlerhood.But if moderate can survive to
  adulthood.

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Chediak-Higashi syndrome

• Degranulation abnormality.
• AR.
• Neutrophils,monocyteslymphocytes contain giant
  cytoplasmic granules.
• In infancy.
• Recurrent pyogenic inf.
• Mild bleeding periphrral neuropathy.
• HSMfever in absence of sepsis.

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DxMx

• Giant granules in neutrophilseosinophils.
• Rx of inf.
• Prophylaxis.
• Ascorbic acid.
• BMT.

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CGD

• NR CGD

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CGD-Clinical presentation

• Recurrent lymphadenitis.
• Skin inf.pneumonia.
• Bact.hepatic abscesses,osteomyelitis at multiple
  sites or small bones.
• Family history of recurrent or unusual catalase
  ve inf.
• Inf.(S. aureus,S.marcensens,Pseudo.
  sp.,Aspergillus sp,C. albicans).

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CGD-

• Chronic inf.
• LAP.
• HSM.
• Chronic purulent dermatitis.
• Restrictive lung dis.
• Gingivitis.
• Hydronephrosis.
• Gastroentestinal narrowing.

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CGD-DxMx

• NBT.
  tetrazoliumO2formazan
• Rx of inf.
• Prophylaxis.
• Gamma IF.

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Myelperoxidase defeciency

• AR.
• The most common.
• MPO catalyzes the production of hypochlorus acid
  in the phagosome.
• Def. Leads to delay in microbial activity.
• MPO defecient neutrophils accumulate more H2O2
• Silent
• Dxperoxidase stain of blood film.
• R xno need.

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