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

[dmvascon at usp.br]

Dear Dr. Spriggs, good morning 

Very interesting case! 
What about his teeth? Are they OK, without periodontal disease? 
The presence of infections due to encapsulated bacteria drives our thoughts to Ig and complement diseases too. 
Despite the lack of descriptions of disease in adults due to defects of NF-kB activation (MyD88 and IRAK-4) you also thought in the possibility of defects in this pathway. Nevertheless, polymorphisms of other TLR molecules (more linked to Gram+ bacteria such as TLR1 and 2) are possible. 
Moreover, defects of the lectin pathway of complement (mainly MASP2 and Ficolin3 deficiencies) are associated with severe sepsis and pneumonias due to encapsulated bacteria. 
Maybe mild defects of phagocytes (such as myeloperoxidase) can account to susceptibility to infections in adult patients, when they develop any other disease that can affect immunity (such as diabetes mellitus and uremia). 
Furthermore, the possibility of a secondary ID, such as those linked to neoplastic diseases such as lymphomas and leukemias can't be ruled out. 

Best regards, 

Dewton 

Dewton de Moraes Vasconcelos, MD, PhD 
University of São Paulo School of Medicine 
São Paulo, Brazil 

----- Mensagem original -----


> De: "Kymble Spriggs" <kymble at me.com>

> Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>

> Enviadas: Terça-feira, 4 de Março de 2014 6:08:26

> Assunto: [cis-pidd] Adult onset, multiple episodes of rapidly

> progressive severe sepsis, with no demonstrable immune abnormality



> 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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