[CIS PIDD] [cis-pidd] Sarcoid granulomatous disease (or lymphoma) and CVID

Tue Aug 26 09:24:45 EDT 2014

Dear Dewton, 

like Jack, I would opt for granulomatous (non-necrotizing) CVID variant though I do not use PET-CT in mine, so cannot comment on SUVs. The calcified pulmonary micronodules would be typical of waxing and waning previously subclinical gCVID variant with previous GLILD/LIP. 

Liver enzymes? If at all high, try liver biopsy, very often positive for granulomas. IL2R often high in blood. Our clinic about 15% of the appr. 100-120 - depending on where You draw the line, die hard min. 100 living - CVIDs have pathologically proven gCVID.

At the same time I would of course check for clinically evolving lymphoma (B symptoms, follow up of LN, inguinal easy to do clinically w/o excessive radiation exposure) but would not be excessively worried about it. From inguinal nodes / urine I would however also check for Ureaplasma and Mycoplasma, Chlamydia, GC a.o.t., with PCR, like You obviously attempted...

Yours, 

Mikko

dos Mikko Seppänen, LKT
Immuunipuutosv-o, HYKS

Mikko Seppänen, MD, PhD, Associate professor/Senior Lecturer 
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
Immunodeficiency Unit
Division of Infectious Diseases
Department of Medicine
Helsinki University Central Hospital
Hospital District of Helsinki and Uusimaa
Aurora Hospital, Ward 4-2 and Outpatient Clinic
P.O.Box 348
FI-00029 HUS, Helsinki
FINLAND
phone +358 9 47175923, fax +358 9 47175945

-----Alkuperäinen viesti-----
Lähettäjä: Dewton Vasconcelos [mailto:dmvascon at usp.br] 
Lähetetty: 26. elokuuta 2014 15:33
Vastaanottaja: CIS-PIDD
Aihe: Re: [cis-pidd] Sarcoid granulomatous disease (or lymphoma) and CVID

Dear Jack, good morning

She has indeed several pulmonary sequelar lesions, as she had several bacterial pneumonias before IVIG replacement.
She has bronchiectasias, some atelectasias and pulmonary micronodules (most calcified) with low SUVs at the PET-CT.
Moreover, the mediastinal lymphnodes are not significantly enlarged and the SUVs are also low.

Thank you very much,

Dewton

Dewton de Moraes Vasconcelos, MD, PhD
Primary Immunodeficiencies Outpatient Unit ADEE3003 Lab. of Medical Investigation Unit 56 University of São Paulo School of Medicine

Routes, John wrote:
> Dewton
> I have seen lymph nodes with SUVs in the teens in patients with 
> multisystemic granulomatous disease-I doubt this is sarcoidosis and 
> would check for pulmonary disease as well Jack
>
> John M. Routes, MD
> Chief, Section of Allergy and Clinical Immunology Professor of 
> Pediatrics, Medicine, Microbiology and Molecular Genetics Department 
> of Pediatrics Children's Hospital of Wisconsin Medical College of 
> Wisconsin
> 9000 W. Wisconsin Ave.
> Milwaukee, WI  53226-4874
> Phone: Office 414-266-6840
> Fax: 414-955-6487 (Clinical)
> Fax: 414-955-6323 (Laboratory)
> Email: jroutes at mcw.edu<mailto:jroutes at mcw.edu>
>
> From: "dmvascon at usp.br<mailto:dmvascon at usp.br>" 
> <dmvascon at usp.br<mailto:dmvascon at usp.br>>
> Reply-To: CLINICAL IMMUNOLOGY LISTSERV 
> <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
> Date: Monday, August 25, 2014 at 5:48 AM
> To: CLINICAL IMMUNOLOGY LISTSERV 
> <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
> Subject: [cis-pidd] Sarcoid granulomatous disease (or lymphoma) and 
> CVID
>
> Dear colleagues
>
> I would like to ask your advice on a case of a CVID patient, female, 24 years-old, born to a consanguineous couple, followed-up in our unit for 12 years.
> She is receiving IVIg replacement since diagnosis with adequate levels and significant clinical improvement of the infectious manifestations, maintaining only chronic sinusitis.
>
> One month ago she came to her IVIG replacement and told about the appearance of an inguinal lymphnode, asymptomatic and without signs of local inflammation.
> This lymphnode was tender to hard and she had two nodes at the other side, 2 cm diameter each.
> We asked for a biopsy (unfortunately the surgeon made an excisional biopsy of the smaller and didn't send to microbiological evaluation), despite the filled forms.
> The histopathological evaluation showed a well formed granuloma without central necrosis, negative for ACB, fungi and HHV8.
>
> A PET-CT showed several lymphnodes in the lower abdomen with high uptake of 18F-FDG (up to 11.5 SUV) in the inguinal region, abdomen and at the Waldeyer ring. It is important to remind that she has chronic sinusitis.
>
> My feeling is that these lymphnodes are affected by a lymphoma and therefore I would need to biopsy another lymphnode.
>
> Have you seen CVID sarcoid lesions with such a high SUV at 18F-FDG PET-CT?
>
> Do you think that it should be safe to try to treat as sarcoid for a while and observe the evolution?
>
> Thank you very much for your opinions.
>
> With my kind regards,
>
> Dewton Vasconcelos
>
> Dewton de Moraes Vasconcelos, MD, PhD
> University of São Paulo School of Medicine
>
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