[PAGID] Lymphopenia and arthritis

[Brian P Vickery]

Thanks for your thoughts, Jason. His IgE is normal and he does not have 
palpable nodes, liver, or spleen. We have not imaged his chest or abdomen, 
and he has not had a slit lamp exam, which is a good idea. Because of the 
poor response to Aldara, the dermatologists just biopsied his diffuse, 
salmon-colored macular rash on Monday and indeed the path demonstrates 
epidermodysplasia verruciformis consistent with HPV infection. 

Brian
___________________
Brian Vickery, MD
Division of Pediatric Allergy & Immunology
Duke University Medical Center
DUMC Box 2644, Durham NC 27710
919.681.2949

"Email should NOT be used for urgent medical issues. The information in 
this electronic mail is sensitive, protected information intended only for 
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Re: [PAGID] Lymphopenia and arthritis

raas0027 
to:
pagid
05/05/2010 05:20 PM


Sent by:
pagid-bounces at list.clinimmsoc.org
Please respond to pagid






Dear Brian,

Interesting case. Lymphocytopenia, impaired cell-mediated immunity, 
arthropathy (can look like JIA) and osteopenia can all be [variable] 
features of sarcoidosis, which can occur at this age.

Not a perfect fit however (e.g. hx low PTH, growth failure, facies) but 
sarcoidosis is pretty heterogeneous.

What about the following:

IgE?

Hepatosplenomegaly?

Intrathoracic (or other) lymphadenopathy?

Skin or eye changes?

Regards,

Jason



On May 5 2010, Brian P Vickery wrote:


>Dear Colleagues: 

>

>I would appreciate your input regarding the following 9 year old boy with 



>lymphopenia and unusual T cell phenotype, growth failure, osteopenia, and 



>a deforming polyarticular arthritis, which is progressive and disabling, 

>affecting the large joints, producing contractures, and requiring 

>methotrexate and intermittent joint injections. The osteopenia was 

>associated with a low PTH which Endocrine has decided was spurious. 

>

>He has a history of recurrent acute / chronic otitis media requiring four 



>sets of tympanostomy tubes and has been treated for sinusitis clinically 

>many times. There is no history of pneumonia, sepsis, meningitis, chronic 



>diarrhea, oral thrush or opportunistic infection. He has verruca plana 

>which are not responding to imiquimod. 

>

>He is small (< 3 %ile for ht & wt) with fine blonde, almost whitish hair, 



>and a hint of a triangularly shaped face but not otherwise dysmorphic and 



>his intelligence is above average. 

>

>The family history is unremarkable. 

>

>Lab findings: 

>-Normal quantitative immunoglobulins with protective diphtheria & tetanus

>-ALC 1000 - 1500; note these numbers and the phenotype were obtained 

prior 

>to starting MTX

>-Lymphocyte enumeration notable for increased B & NK cells, decreased 

>alpha/beta and increased gamma/delta T cells, and normal RTEs: 

> 

>CD3

>45.8%

>453

>CD4:CD8 

>0.82

>

>Ti alpha/beta

>25.5%

>252

>Ti gamma/delta

>19.0%

>188

>CD16

>20.4%

>202

>CD56

>19.6%

>194

>CD19

>29.5%

>292

>CD20

>31.9%

>316

>CD45RO

>16.9%

>77

>CD45RA

>56.7%

>257

>45RA+/62L+

>27.2%

>123

>

>-Impaired proliferation to PHA (44,000), and ConA (12,000), and 

borderline 

>response to PWM (44,000)

>-No 22q11 deletion

>-RMRP sequencing normal

>-46XY with normal Affymetrix SNP 6.0 microarray

>-Sweat chloride normal with negative CFTR mutation screen

>-IGF-1 and IGFBP-3 low

>-RF neg, HLAB27 neg, ANA 1:160, speckled pattern

>

>I would be grateful for any diagnostic or therapeutic suggestions. 

>

>Best regards, 

>Brian 

>___________________

>Brian Vickery, MD

>Division of Pediatric Allergy & Immunology

>Duke University Medical Center

>DUMC Box 2644, Durham NC 27710

>919.681.2949

>

>"Email should NOT be used for urgent medical issues. The information in 

>this electronic mail is sensitive, protected information intended only 

for 

>the addressee(s). Any other person, including anyone who believes he/she 

>might have received it due to an addressing error, is requested to notify 



>the sender immediately by return electronic mail, and to delete it 

without 

>further reading or retention. The information is not to be forwarded to 

or 

>shared unless in compliance with Duke Medicine policies on 

confidentiality 

>and/or with the approval of the sender."

>


-- 
Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N
Suite 110
Plymouth, MN 55446

(Phone) 763.577.0008
(FAX)   763.5770192


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