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

[CIS-PIDD]

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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