[CIS PIDD] [cis-pidd] Adult onset, multiple episodes of rapidly progressive severe sepsis, with no demonstrable immune abnormality

[Kymble Spriggs]

Thankyou for your consideration regarding the further investigation for an eitiology of this gentleman's recurrent infections:

40yo Caucasian man suffering multiple separate rapidly progressive severe episodes of sepsis.

Acute History:
-  First occurring 2 years ago - resulting in ICU admission and leg and fingertip amputation.
--  Confirmed to be pneumoccocal sepsis
-  Two further episodes of severe pneumonia treated at external hospitals
-  Last year suffering similar rapidly progressive sepsis – patient becoming unconscious within hours of feeling unwell.  Found by partner & taken to hospital.
--   Later confirmed to be Haemophilus Influenzae (non-typable, biotype IV)
-  Subsequently remaining well on prophylactic antibiotics (amoxicillin)

Past History:
- No other previous significant infection history – without significant illness until recently.
- Tonsillectomy in childhood & mild eczema and bronchiolitis.
- Possible “bruised spleen” during traumatic accident in childhood.  Anatomically present on CT scan.  No howell-jolly bodies on blood films
 
No significant family history or consanguinity.  Currently well offspring

Laboratory Results:
- Full blood count unremarkable
- Normal Lymphocyte Subsets (including normal CD3+, CD4+, CD8+, CD19+ and CD16+/56+)
- Normal IgG, IgA, IgM, IgE
- Excellent dynamic pneumococcal antibody responses to Pneumovax23 – high titres to 14/15 serotypes
- Good Haemophillus influenza type B antibody levels
- HIV Negative
- Normal functional complement studies (CH100, AH50)
- Normal CD62 ligand shedding (to LPS and PMA)
- Normal memory B cells/ B cell subsets

Are there any other investigations that could be performed on this gentleman, to try and clarify his underlying diagnosis?

Kind Regards,

Dr Kymble Spriggs
MBBS(Melb), MCRP(UK), DTMH(Lon), FRACP

Clinical Immunology & Allergy
Royal Melbourne Hospital












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