[PAGID] Patient with lymphopenia

[Berger, Melvin]

Stim tests shoulod be standardized to the number of cells, so you will need a greater volume of blood. The lab should be informed in advance based on the latest CBC and diff, but they should be able to purify the number of cells they need. It is often the case in DiGeorge's that the number of T-cells is low, but the mitogen responses are normal. By the way, does this patient have any features of DiGeorge's at all ? That would certainly be one consdition which could give you this picture.
 
Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Jason Raasch, MD
Sent: Sun 4/20/2008 10:02 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Patient with lymphopenia



Mel,

 

No.  A technical question I wanted input on is whether that degree of lymphopenia would result in falsely low results with a standard sample.  Would I need to draw a [relatively] large volume of blood and 'concentrate' her lymphocytes prior to the stim tests?

 

--jason

 

Jason P. Raasch, MD

Midwest Immunology Clinic

 

West Health

2805 Campus Dr, #215

Plymouth, MN 55441

 

Telephone: 763.577.0008

FAX: 763.577.0192

e-mail: raas0027 at umn.edu

________________________________

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Berger, Melvin
Sent: Sunday, April 20, 2008 8:53 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Patient with lymphopenia

 

Have mitogen/antigen stim tests been done ?

 

Melvin Berger, M.D., Ph.D.

Professor of Pediatrics and Pathology

Case Western Reserve University

phone 216 844 3237

 

Director, Jeffrey Modell Center for Primary Immune Deficiencies

Division of Allergy-Immunology

Rainbow, Babies and Children's Hospital

University Hospitals of Cleveland

RB&C Rm 504, MS 6008B

11100 Euclid Ave.

Cleveland, OH 44106

 

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Jason Raasch, MD
Sent: Sun 4/20/2008 9:42 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

 

Visit us at www.UHhospitals.org.

The enclosed information is STRICTLY CONFIDENTIAL and is intended for the use of the addressee only. University Hospitals and its affiliates disclaim any responsibility for unauthorized disclosure of this information to anyone other than the addressee.

Federal and Ohio law protect patient medical information, including psychiatric_disorders, (H.I.V) test results, A.I.Ds-related conditions, alcohol, and/or drug_dependence or abuse disclosed in this email. Federal regulation (42 CFR Part 2) and Ohio Revised Code section 5122.31 and 3701.243 prohibit disclosure of this information without the specific written consent of the person to whom it pertains, or as otherwise permitted by law.



Visit us at www.UHhospitals.org.

The enclosed information is STRICTLY CONFIDENTIAL and is intended for the use of the addressee only. University Hospitals and its affiliates disclaim any responsibility for unauthorized disclosure of this information to anyone other than the addressee.

Federal and Ohio law protect patient medical information, including psychiatric_disorders, (H.I.V) test results, A.I.Ds-related conditions, alcohol, and/or drug_dependence or abuse disclosed in this email. Federal regulation (42 CFR Part 2) and Ohio Revised Code section 5122.31 and 3701.243 prohibit disclosure of this information without the specific written consent of the person to whom it pertains, or as otherwise permitted by law.
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