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

Fri May 12 14:16:24 EDT 2017

Patricia,

Complement deficiency considered?

H. influenzae isolated with sputum or BAL with EVERY episode?  IN other words, is this absolutely without a doubt secondary to bacterial infection or could it be inflammatory pulmonary disease?

I am in agreement with Timi.  I will go out on a limb and say I am an 'IgM deficiency' skeptic.  Whenever there is an ISOLATED finding of 'low' IgM (i.e. the only lab abnormality noted), I suspect there is either a yet-to-be-identified immunologic deficiency/dysregulation process and/or a secondary cause (e.g. in this patient aspiration, vasculitis, etc. as has been proposed).  In other words, I feel there has to be more to the story than just low serum IgM.  This is my personal working hypothesis.

It is possible immunoglobulin would help, although it would not prove the low IgM is the problem given the anecdotal reports of IVIG/ScIg benefit in sinopulmonary disease not directly related to primary immune deficiency (e.g. asthma, COPD, CF, etc.).  If the patient continues to have recurrent pneumonia despite your current management AND you can get immunoglobuin replacement  covered, it may be worth a try.

________________________________
From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Friday, May 12, 2017 12:13:47 AM
To: CIS-PIDD
Subject: VS: [cis-pidd] selective IgM deficiency

Hi

Have you considered gastroesophageal reflux?  (the bypass surgery 2012)

In the literature selective IgM deficiency has rather mild phenotype, mainly allergy.

Best regards

Timi Martelius
Infectious Diseases
Helsinki University Hospital

Lähettäjä: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] Puolesta CIS-PIDD
Lähetetty: 11. toukokuuta 2017 22:51
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net>
Aihe: [cis-pidd] selective IgM deficiency

Dear colleagues,

 I am Patricia Roessler, a chilean Clinical Immunologist. .
I would like your opinion about a clinical case, if it is possible.

The patient is a male of 50 years, previously healthy until 2015 when he had a mild influenza H1N1 infection. Then on, he had have 6 episodes of pneumonia without fever but with leucocytosis, neutrophilia and elevated ESR and CRP. He was treated with oral antibiotics with regular response so he needed intravenous antibiotics to recover from this pneumonias.
The infections were always in the same lobe ( inferior right lobe). He had a Fibrobronchoscopy with BAL in November 2016. There was hypercellularity with 95% of neutrophils and Haemphylus Influenzae grew in cultures. The biopsy was inespecific.
In Lung Images no anatomic defect could be detected but as the pneumonias were always in the same place a lobectomy was done in March 2017.
In Abril 2017 he had a pneumonia, now in the left lung, with fever and similar characteristics in general labs than previous episodes.

His infectious history was anodyne, except for recurrent tonsillitis in infancy ( he went a tonsillectomy). He refers no sinus infections, although in a a PET CT done in this hospitalization a sinusitis was described.

He had a gastric by-pass surgery because of obesity and diabetes in 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 evaluation with serum electrophoresis and immunofixation being normal, and a PET CT without signs of an occult neoplasm. The patient denied a Bone Marrow biopsy.

A malabsortion syndrome was diagnosed (low carotene, very low D vitamins and zinc levels, normal albumin ) and assumed to be secondary to his by pass.
Celiac disease serology is negative

I think that this patient has a primary selective IgM deficiency but I don´t know well how to treat him.
- Do you think he needs more immunologic studies ( B memory cells and anti Haemophylus influenza antibodies are in course, antibodies to protein antigens have not been measured).

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

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

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

Thank you very much and I hope you can help me

Best regards,

Patricia

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