[CIS PIDD] [cis-pidd] Patient with low IgM

[CIS-PIDD]

Hi!

The person posting this gave us no clue who he/she is? Could you please always sign?

According to the levels given, this patient has low IgG, low IgM and borderline low IgA, thus the title of this case is somewhat misleading? He sounds like a CVID candidate/in the making, but there are quite a few secondary causes potentially possible.

What is his nutritional (protein nutrition) status?
What are the Ig levels when measured at least 3 months after the previous invasive infection?
Calprotectin levels in stools?
Diarrhea due to exocrine pancreatic insufficiency?
Urine albumin/protein loss?
Fecal antitrypsin levels?
Has monoclonal gammopathy been excluded?

In alcohol hepatopathy, one would expect to see high Ig levels (esp IgA and IgG).

What was the logic behind measuring anti-PnP as early as in 2 weeks-  I did not quite get that?
Was the vaccine given very early after a septic episode?

All the cases of presumed cellulitis after Pneumovax I have seen (n about 6-7) were all sterile- though looked a lot like bacterial, and though IV antibiotics were given, skin was almost back to normal in just 3-4 d, which in bacterial cellulitis just does not happen. And all these patients had very robust anti-PnP levels 4 weeks after vaccine, they were thus rather hyperresponsive (which one can be even with panhypogamma).

I would want to see the anti-PnP titers and Ig levels  now...?

A single or even recurrent bacterial pneumonia due to alcohol  abuse is not rare.

Thus ....this case left me with more questions than answers...

Isolated low IgM- tend to agree with Soheil, far too common, most of the patients too healthy...

Sorry for maybe just confusing you more?

Mikko


Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)

Chief, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 20.10.2015 kello 0.26:

Were Pneumovax and influenza vaccine administered in opposite sides or in the same arm? If the former were the case, you could isolate the culprit by laterality, and focus your attention on the culprit.

I have seen a few Arthus-type reactions in patients who have had a high titers of preexisting antibodies to one of the vaccine components prior to vaccination. They display swelling, redness warmth and tenderness, and are often misdiagnosed with infectious cellulitis and subjected to unnecessary antibiotics. Severe infection caused by influenza virus, and pneumonia in an apparently immunocompetent host shortly prior to vaccination would be consistent with this assumption.

I am not certain, if a definitive pathologic value can be assigned to isolated low IgM. I have stumbled into patients with low IgM and in them I could not find a single condition that could explain the low levels of IgM, which could be generalized to different individuals who displayed it. I am not sure, if this is of any help. I hope others will be able to share their insight, so that we can utilize collective knowledge and experience in managing similar cases.

Soheil Chegini, M.D.
Exton Allergy & Asthma Associates
656 West Lincoln Hwy.
Exton, PA 19341
Phone: (610) 269-3066
Fax: (610) 269-8615


________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Sent: Monday, October 19, 2015 10:14 AM
Subject: [cis-pidd] Patient with low IgM

Hello, I was hoping you could provide some insight for a patient that I am seeing. Thank you in advance for your help.


The patient is a 41 year old male who was referred to me after he developed left arm cellulitis after a pneumovax and flu vaccination in September 2015. He required 1-2 days of IV antibiotics and completed an oral antibiotic regiment.  He also developed pneumonia and sepsis in June 2015 after testing positive for the flu vaccine. He required intubation at that time and was hospitalized for a few weeks.

He has no other history of recurrent infections including sinusitis or pneumonia. He was hospitalized in 2012 for alcohol withdrawal. He has a history of moderate etoh intake and he was told that he had to be treated for alcohol withdrawal during the June 2015 hospitalization as well.
He states he no longer drinks alcohol.

He does not have any other significant medical history. He does not have a history of asthma or eczema. He was born in the US.  He does not take any medications but did receive vivitrol IM injections for 8 months in 2012 for etoh abuse.  He is of Irish and Sicilian descent; he is married with three young healthy children. His mother passed away from uterine cancer and his father had HTN and passed away from heart disease.  He has two sisters, one who is in her 40s and has a “positive ANA.” He has a maternal great aunt with lupus.  He is self-employed in real estate.

His labs are notable for a low IgM.

WBC 14.8 (range 4-10), normal Hg, Hct and Platelets, normal differential
BMP/LFTs normal

IgG: 494 (range 700-1600 mg/dL) --> repeat  605 mg/dL (range 700-1600 mg/dL)
IgM: 18 (range 40-230 mg/dL) --> repeat 19 (range 40-230 mg/dL)
IgA: 74 (range 91-414 mg/dL) --> repeat 92 (range 91-414 mg/dL)
IgG subclass: IgG1: 333 mg/dL (range 422-1292 mg/dL), IgG2: 230 mg/dL (117-747 mg/dL), IgG3: 41mg/dL (41-129 mg/dL), IgG4 16 mg/dL (1-291 mg/dL)
IgE 14 IU/mL (range 0-100)


HIV negative
He is blood group A; he has a positive Isohemagglutinin B titer 1:8.

C3 normal, C4 40 mg/dL (9-36 mg/dL)

He has protective titers for diphtheria, tetanus, varicella, rubella and H. Influenzae type B.
He does not have protective titers for n. meningitides, mumps, rubeola and only has 8/23 protective titers for pneumococcus (2 weeks after pneumovax).


He has negative titers for HbsAg, HepBs IgM/IgG, HepB core IgM, HepA IgM, HCV IgG.

A lymphocyte panel including absolute values for CD3: 1452 (range 622-2402); CD4: 796 (range 359-1519); CD8 557 (109-897), CD19 348 (12-645) is normal.

He is ANA negative.

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