[CIS PIDD] [cis-pidd] selective IgM deficiency

[CIS-PIDD]

Dear colleagues,


 
 I am Patricia Roessler, a chilean Clinical Immunologist. .

I would like your opinion about a clinical case, if it ispossible.


 
The patient is a male of 50 years, previously healthy until 2015when he had a mild influenza H1N1 infection. Then on, he had have 6 episodes ofpneumonia without fever but with leucocytosis, neutrophilia and elevated ESRand CRP. He was treated with oral antibiotics with regular response so heneeded intravenous antibiotics to recover from this pneumonias.

The infections were always in the same lobe ( inferior rightlobe). He had a Fibrobronchoscopy with BAL in November 2016. There washypercellularity with 95% of neutrophils and Haemphylus Influenzae grew incultures. The biopsy was inespecific.

In Lung Images no anatomic defect could be detected but as thepneumonias were always in the same place a lobectomy was done in March 2017.

In Abril 2017 he had a pneumonia, now in the leftlung, with fever and similar characteristics in general labs than previousepisodes.


 
His infectious history was anodyne, except for recurrenttonsillitis in infancy ( he went a tonsillectomy). He refers no sinusinfections, although in a a PET CT done in this hospitalization a sinusitis wasdescribed.


 
He had a gastric by-pass surgery because of obesity and diabetesin 2012 ( both of this conditions reverted until now)


 
He has frecuent diarrheas last year.


 
In Immunologic studies he has:


 
November 2016: 

IgG 1233 

IgA 436 (mild elevation)

IgM 31 (low) 


 
Normal IgG subclasses


 
80% of anti pneumococcal antibodies were in protective titles(never vaccinated) 


 
April 2017: 

IgG 919

IgM 22

IgA 327 


 
Lymphocytes subpopulations were all normal.

 

As an IgM deficiency was found he went an hematologic evaluationwith serum electrophoresis and immunofixation being normal, and a PET CTwithout signs of an occult neoplasm. The patient denied a Bone Marrow biopsy.


 
A malabsortion syndrome was diagnosed (low carotene, very low Dvitamins and zinc levels, normal albumin ) and assumed to be secondary to hisby pass. 

Celiac disease serology is negative 


 
I think that this patient has a primary selective IgM deficiencybut I don´t know well how to treat him.

- Do you think he needs more immunologic studies ( B memorycells and anti Haemophylus influenza antibodies are in course, antibodies toprotein antigens have not been measured).


 
- Do you think he can benefit from IVIG? (I have read thatpatients with selective IgM deficiency and low anti pneumococcal antibodies canhave a good responde but is not the case of this patient)


 
- Do you think that Pentaglobin could help (I have no experiencewith this IVIG)?


 
Meanwhile he is receiving conjugated pneumococcal an H.Influenza vaccines and I have put him in prophylactic antibiotics ( dailysulphametoxazol/thrimetropin) 


 

 
Thank you very much and I hope you can help me


 
Bestregards,


Patricia 
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