[PAGID] Patient with lymphopenia

[Routes, John]

Jason
I had a very similar patient ---Middle aged female with  a soft hx of SLE in
the past---was ANA negative during my eval  (but low positives in the past)
and did not have symptoms cw active SLE ---persistently low CD4/8 T cell
counts (< 200 total)--- DTH was negative but mitogen/Ags were normal----nl
Igs, and normal specific Abs following immunization--She had severe verruca
vulgaris over the heels of both feet--- --warts resistant to numerous types
of therapy but did respond nicely to imiquimod. A screen for anti-lymphocyte
Abs was strongly positive------she has never had opportunistic infections
and continues to have persistently low T cell counts (for several
years)-----recently developed ITP, but nothing else-----
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: 414-456-4802; 456-4803
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu
 




From: "Jason Raasch, MD" <raas0027 at umn.edu>
Organization: University of Minnesota
Reply-To: <pagid at list.clinimmsoc.org>
Date: Sun, 20 Apr 2008 20:42:10 -0500
To: <pagid at list.clinimmsoc.org>
Conversation: Patient with lymphopenia
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 20¹s 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 1970¹s 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
Children¹s 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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