[CIS PIDD] [cis-pidd] Hypergammaglobulinemia with low CD4

Mon Nov 7 15:49:45 EST 2016

We have seen hypergamm,  elevated B12, sinus infections with incomplete polysaccharide responses and T cell lymphopenia in some of our ALPS patients. they were also very atopic with superficial skin infections. did you check DNTCs? Splenomegaly? LAD?

Mary Hintermeyer
Advanced Practice Nurse Practitioner
Primary Immune Deficiency Program
Children's Hospital of Wisconsin
Jeffrey Modell  Diagnostic and Research Center for Primary Immunodeficiencies
PH: 414-266-6293  FAX: 414-266-1649

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Monday, November 07, 2016 2:27 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] Hypergammaglobulinemia with low CD4

Dr. Tichenor thanks for your input
Yes he did receive prevnar -7
No pneumovasx
We gave pneumOvax and requested for  titers-23 serotype
All IgG sub classes were very high
ESR range from 20-60( probably due to high Immunoglobulins)
No polyps on exam  no history
CF testing done due to lack of any clinical evidence
There is questionable history of "boils"
No cultures were performed due to vague history of "sinus infections"
Seen by hematology who cleared this patient after their work up for anemia . Other iron profile  was normal.
No B-12  supplements
Diphtheria titers pending
We are still wondering if it is some sort of connective tissue disorder due  to normal leucopenia/lymphopenia , CRP and high gammaglobulins
Not sure why CD-4 count is low ( debating about sending lymphocyte stimulation test with PHA)
Could it be  CGD due to questionable history of boils ?

Many thanks
Ejaz

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Sunday, November 06, 2016 9:15 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] Hypergammaglobulinemia with low CD4

Did the child receive Prevnar-7 and recent Pneumovax?  Were repeat titers done ( suggest 23 serotypes). Were the subtypes normal?  How high was the ESR?  Were there any polyps?  Was screening done for CF?  Were endoscopically directed cultures performed?  What was the balance of the CBC results including RBC and WBC morphology?  Was the anemia and elevated Fe worked up completely?  Folate levels? Any B12 supplements?  Diphtheria and Haemophilus titers?

Sincerely,

Wellington S. Tichenor, M. D.
642 Park Avenue
New York, New York 10065
212 517-6611
wtichenor at sinuses.com<https://urldefense.proofpoint.com/v2/url?u=https-3A__register.concentric.com_home_apps_mail_mbox-5Fcompose.cgi-3FpTo-3Dwtichenor-40sinuses.com&d=DgMF-g&c=X2IGR6v8ax_mLhSmU1r3Aw&r=re9XgkJJQL9cy7kWhnyy47GAzX9cHmXjpMXZICZRq_U&m=64g7JhDxD-uSrmkfVUnn0TnaY3o8vGGsOO5igo3g3wY&s=ANedbMm2eyggF-aakWFlNG768huLfW-ydgefBeZjEP8&e=>

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From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Sunday, November 06, 2016 10:09 AM
To: CIS-PIDD
Subject: [cis-pidd] Hypergammaglobulinemia with low CD4
Dear colleagues and experts:  I would like to obtain your input on an 8 years old male child with history of fatigue, recurrent infections (non-specific URIs, otitis and "sinus infections") treated with antibiotics 2-4 times/year.
History of anemia, nonspecific joint pain, lack of concentration and focusing. No fevers
No other abnormal symptoms
FH +ve for atopic issues and sinus issues. No immunodeficiency
Normal exam multiple times (no eczema, lymphadenopathy, hepatosplenomegaly)
Labs:
persistent leukopenia (WBC 2.8-5.5)
Anemia-Hb 10-102, High ferritin -221-289, High vitamin B level >1500-2000
Elevated ESR normal CRP
Immunoglobulins----
IgG-1583-2170
IgA-417-522
IgM-55-78
Low lymphocyte count ----1100-1730
CD4---280-417
CD8----317-405
CD-19-310-420
NK cell( cd3 +) 92-98
Normal tetanus titers
Only 4/14 protective pneumococcal titers (pre vaccine)
Normal ANA profile (negative auto antibodies panel)
Negative for HIV
Normal thyroid functions
Summary; 8 years old fatigue, joint pains nonspecific upper respiratory infections with hypergammaglobinemia, leukopenia, lymphopenia, low CD4 count, high ferritin and elevated B12
Any guidance /input would be appreciated
Thanks

Ejaz Yousef MD
Nemours Children Clinic
Jacksonville, Fl

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