[PAGID] Patient with lymphopenia

[Marc Riedl]

Jason,

 

We've seen a few such patients here as well.   I agree with many of the
previous suggestions:   

-          We typically perform in vivo DTH testing as well as in vitro
proliferation assays

-          We have identified ADA deficiency in one case

-          However, most of the time we end up with a diagnosis of
"idiopathic lymphopenia" having ruled out other potential causes

The good news is that in my experience, the vast majority of these patients
do well clinically without serious life-threatening infections.

 

Regards,

 

Marc

 

Marc Riedl, M.D., M.S.
Assistant Professor of Medicine
Section Head, Clinical Immunology and Allergy
UCLA - David Geffen School of Medicine
10833 Le Conte Ave, 37-131 CHS
Los Angeles, CA  90095-1680 
Tel 310.206.4345  Fax 310.267.0090 

 

 

 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jason Raasch, MD
Sent: Sunday, April 20, 2008 6:42 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] Patient with lymphopenia

 

Please offer your insight into this 53 year old woman with persistent
verruca plantaris (plantar warts) and severe lymphopenia.  Consider the
following questions as you review the case:

 

First note flow cytometry (presented in absolute numbers, gated on CD45):  

 

Total CD3 lymphocytes: 53 per mm3 (reference 1064-1672)

CD3+CD4+: 10 (627-1102)

CD3+CD8+: 38 (247-741)

CD4:CD8 = 0.27

CD19:  93 (95-418) 

CD56+16+: 93 (95-494)

 

At first glance, whether primary or acquired, this is striking.  Now
consider her clinical history:  She was well from birth through adolescence.
Varicella at age five; shingles at age 19.  Seizures at age 23; on
Phenobarbital for 10 years this was then discontinued and she has had no
further neurologic events.

 

By her late 20's she began having persistent verruca vulgaris on hands,
knees, pre-tibial area and feet.  Over the last 30 years has failed OTC
therapies as well as excision, laser ablation, cryotherapy, topical and oral
retinoids and localized bleomycin.

 

Variably positive ANA (up to 1:320, but often undetectable) starting in her
30s.  Other than intermittent arthralgias, no other symptoms.
Hypothyroidism diagnosed at age 51 (thyroglobulin and thyroperoxidase
antibodies undetectable).

 

Lymphopenia brought to her attention at age 52 (WBC 4,000/mm3; ANC=3,200;
ALC=240).  Medical record review actually demonstrates same findings as far
back as 1970's and persistently each decade thereafter, suggesting a
long-standing finding.

  

Review of systems completely unremarkable except for the above, as well as
mild depression and hypothyroidism.  No GI symptoms.  No history of
recurrent infection.  No history of heart disease or abnormal facies.
Patient reports unremarkable family history.

 

Over the last 18 months the following have been UNREMARKABLE: electrolytes,
ALT/AST, serum albumin and protein, B12, folate and iron levels, serum
electrophoresis/immunofixation, quantitative immunoglobulins,
tetanus/diphtheria, pneumococcal and varicella titers, isohemagglutinins,
rheumatoid factor, dsDNA, CCP antibody, CRP, ESR, C3, C4, CH50, TSH and T4.
Stool alpha-1 antitrypsin normal.  HIV ELISA X 2 negative.  FANA 1:40 with
homogeneous pattern.  

 

INTERESTINGLY, this patient reports that her HEALTHY adolescent daughter was
incidentally found to have just as severe a lymphopenia AND neutropenia.

 

In summary this is a fairly health woman with a striking lymphopenia (that
most likely has been present over the last 30+ years), persistent verruca
vulgaris and intermittently positive ANA.  A HEALTHY daughter has
lymphopenia and neutropenia and has not had evaluation.

 

So the questions:

1.	Who has seen cases like this?
2.	Why has she had such a benign course despite such a lymphopenia?
3.	To what extent should a diagnosis be pursued?  In her daughter?

 

I am interested in any comments.

 

Have a great week.

 

--jason

 

Jason P. Raasch, MD

Children's Hospitals and Clinics of Minnesota

 

Midwest Immunology Clinic

2805 Campus Dr, #215

Plymouth, MN 55441

 

Telephone: 763.577.0008

FAX: 763.577.0192

e-mail: raas0027 at umn.edu

 


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