[CIS PIDD] [cis-pidd] Patient with CVID, inguinal lymphadenopathies and lymphedema

[Patar]

Hi I  like to ask your opinion about this case:

Man
40 years old
In 2002 he presented with bilateral inguinal lymphadenopathies, malaise, without fever or elevation of inflamatory parameters. Biopsy of inguinal lymphadenopathies: suppurated granulomas without any microbiological agent found.

>From then on, we has had intermitently periods of inguinal pain, diarrhea and arthralgias of  hands and axial spine ( without synovitis).

In 2007 he had another inflammation of inguinal adenopathies. Biopsy describes a polyclonal lymphocitic hyperplasia.
He received prednisone at high doses and Azulfidine without clinical response.
2008: the diagnosis of CVID was made based in a midly low level of IgG (600 aprox) and poor response to polysacharides and protein vaccines.
He has no history of recurrent infections.
Familiar medical history: father died of Non Hodking lymphoma and had a history of recurrent sinopulmonary infections.

>From then on he received monthly infusions of IVGG with improvement of arthralgias. However we continued having inflammation in inguinal lymph nodes with appearence of mild lymphedema in lower extremities ( confirmed by isotopic lymphocintigraphy).

He had received metothrexate and hidroxyclorochine ( up to 4,5 mg/ kg) with persistence of inflammatory inguinal adenopathies and intermitent autolimited periods (he uses naproxen) of arthralgias and diarrhea ( wihout elevation of inflammatory parameters).
He has normal scanner of abdomen, pelvis and thorax. Normal upper endoscopy and colonoscopy
He is very worried about the eventual progression of lower extremities lymphedema.

¿ what would you offer him?

Kind regards

Patricia Roessler
Alergia e Inmunología
Chile


El 13-06-2013, a las 21:45, "Leiding, Jennifer" <jleiding at health.usf.edu> escribió:


> Hi all:

> 

>  

> 

> I am following a little 6yo boy with chronic Giardia infection starting at age 4. He has intermittent symptoms of abdominal pain and diarrhea or loose stools. He has been treated with prolonged courses of metronidazole, paramomycin, and Alinia without effect. Endoscopy and colonoscopy were normal with the exception of Giardia on biopsy specimens from the colonoscopy.  He is thriving well and not losing weight.  There is no history of major infections  but does have frequent URI’s. Other parasitic infections, cryptosporidium, and C. diff were ruled out. Vitamin deficiencies associated with chronic giardiasis were also ruled out.

> 

>  

> 

> He lives in an urban area and no one else in the family has been infected. He has an older brother who is healthy. His family had their water checked by the city and was normal with no parasites or other enteric organisms. 

> 

>  

> 

> CMP is normal

> 

> CBC – normal with ALC 2700, ANC 3400 AEC 270

> 

> Diphtheria, tetanus, pneumococcal titers normal.

> 

> IgG 832, IgA 94, IgM 87, IgE 178

> 

> Absolute CD3 1707, CD4 1178, CD8 418, CD56 154, CD19 518

> 

> Normal endoscopy and colonoscopy other than Giardia found on biopsy slides

> 

> Vitamin C 1.62 vitamin A 30 folate 19.4

> 

>  

> 

> Does anybody have any ideas about further workup or management?

> 

>  

> 

> Thanks,

> 

> Jenny Leiding

> 

> 

> Jennifer Leiding, MD

> Assistant Professor

> University of South Florida

> Department of Pediatrics

> Division of Allergy, Immunology, and Rheumatology

> Children’s Research Institute

> 140 – 7th Avenue South Box 9680

> St. Petersburg, Florida 33701

> Phone: 727.553.3519

> Fax: 727.553.1295

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