Le ukocyte Qualitative Disorders

1. Leukocyte Qualitative Disorders Presented by:Dr Akram Sa’adeh Moderator:Dr Yousef Abu Osba

4. Neutrophil rolling

7. Evaluation for phagocytic cell disorder • Tow or more systemic bacterial inf. • Three or more serious respiratory or documented bacterial inf. • Inf. Occurring at unusual sites. • Inf. with unusual pathogens. • Inf. With common childhood pathogens but of unusual severity.

8. Disorders of neutrophil function

9. Job syndrome • Disorder of chemotaxis. • AD. • Increase IgE LEVEL. • Recurrent Staph. Abscesses(cold abscesses). • Course features. • Osteoporosis. • Recurrent pneumonias.esophagitis&meningitis

10. 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.

11. Job syndrome

12. Job syndrome

13. Job syndrome

14. Job syndrome

15. Job syndrome-Dx&Mx • High IgE, mild increase in IgD. • NR WBC,eosinophils up to 50%. • Soft tissue &syst. Inf. :broad spectrum AB. • Fungal inf. • Gamma IF. • Prophylaxis.

16. 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.

17. LAD-Dx&Mx • Flow cytometry &measurement of surface CD11/CD18. • Supportive. • Prophylaxis. • BMT. • Prognosis:often not surviving beyond toddlerhood.But if moderate can survive to adulthood.

18. Chediak-Higashi syndrome • Degranulation abnormality. • AR. • Neutrophils,monocytes&lymphocytes contain giant cytoplasmic granules. • In infancy. • Recurrent pyogenic inf. • Mild bleeding &periphrral neuropathy. • HSM&fever in absence of sepsis.

19. Dx&Mx • Giant granules in neutrophils&eosinophils. • Rx of inf. • Prophylaxis. • Ascorbic acid. • BMT.

20. CGD NR CGD

23. 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).

24. CGD- • Chronic inf. : • LAP. • HSM. • Chronic purulent dermatitis. • Restrictive lung dis. • Gingivitis. • Hydronephrosis. • Gastroentestinal narrowing.

25. CGD-Dx&Mx • NBT. tetrazolium……O2……formazan • Rx of inf. • Prophylaxis. • Gamma IF.

26. 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 • Dx:peroxidase stain of blood film. • R x:no need.

27. الحق هناك ينادينا وطيور الدوح تغنينا و دماء شهيد ملتحد فى السفح تراب فلسطينا

28. وسنرجع يوما يا وطني • وسنغرق فيك امانينا • يا وطني كنت لنا دنيا • وخلاصك صار لنا دينا

29. راجعين