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    NK Immunodeficiencies NK Immunodeficiencies Presentation Transcript

    • Gaslini Advanced Course in Immunology
      Genoa, July 9-13, 2007
      Investigation of isolated Natural Killer cell deficiencies in children with herpetic encephalitis.
      Filippo Fassio
      Immunology and CellularTherapiesUnit,
      University ofFlorence
    • NK and Herpetic Infections…
      1989 – Biron: Severe herpes virus infections in an adolescent without natural killer cells.
      N Engl J Med 1989;320:1731–5
      1996 - de Vries: Identification of an unusual Fcγ receptor IIIa (CD16) on natural killer cells in a patient with recurrent infections.
      Blood 1996; 88: 3022-3027
      2003 - Levy: Disseminated varicella infection due to the vaccine strain of varicella-zoster virus, in a patient with a novel deficiency in natural killer T cells.
      J Infect Dis. 2003;188(7):948-53
    • Isolated NK Deficiencies: Classification
      Orange JS. Human natural killer cell deficiencies. Curr Opin Allergy Clin Immunol 2006
      Absolute NK Deficiency
      lack of CD56+ cells, which comprehend both classic NK cells and NKT cells
      Classic NK Deficiency
      lack of classic CD3-CD56+ NK cells
      Functional NK Deficiency
      presence of NK cells with functional deficiency, most typically of cytotoxicity
      NK + NKT cells
      NK cells
      CD16 polymorphism
    • Aim of the study
      We investigated a small series of children affected by HSV infection and encephalitis to evaluate clinical and epidemiologic importance of NK deficiencies as a predisposing factor for increased susceptibility and severe course of herpetic infections.
    • Patients
      5 consecutive children with HSV encephalitis were enrolled at Mayer Pediatric Hospital in Florence so far…
      Diagnosis was based upon PCR documentation of HSV in CSF
      Only 1 child (no. 2) was lymphopenic
      No deficiencies of other branches of immunity
    • Results/1 – ‘Classic’ NK and ‘NKT’ Cells
      *: Low intensity of fluorescence
      *
      B73.1 MoAbbindingto CD16 isimpairedbypolymorphisms in position 230
      Cases no. 1, 2 and 4:
      Absolute NK deficiency
    • Results/2 – CD16 Polymorphisms
      Wild type (cases no.1, 2 and 4)
      230 T/T
      Heterozygoticpolymorphism (no.3)
      230 T/A
      Homozygoticpolymorphism (no.5)
      230 A/A
      Cases no. 3 and 5:
      Functional NK deficiency
    • Conclusion/1
      • Numeric and/or functional deficiencies of NK cells were present in all our patients affected by severe herpetic infection with encephalitis
      • NK cell deficiency was isolated, as other branches of immunity were working properly, in allbutoneofthem
      • Important reduction of T cells expressing NK-associated receptors (‘NKT’ cells) was also present in all the children with numeric NK cell deficiency
    • Conclusion/2
      • These findings emphasize the role of NK-related immunodeficiencies in determining increased incidence and worse outcome of viral infections in humans, in agreement with previously published reports.
      • NK cell populations are still rarely investigated in these conditions
      • Despite the lack of an actual therapeutic approach, being able to identify higher risk patients would allow avoiding voluntary exposure to infected contacts, prompt instauration of anti-viral therapies in case of infection and higher degree of attention in those conditions, such as pregnancy or concomitant use of immunosuppressive drugs, in which risk of severe injury is elevated
    • Thank You!