[CIS PIDD] [cis-pidd] 2 yo w/increased double negative t-cells and mesenteric adenopathy

[O'Connell, Amy]

I cannot find that it says whether EBV status was checked in your summary.
I also wonder about Castleman's disease.  Multicentric disease is even more rare in children but you can have an associated eosinophilia, and if the biopsy was a needle or core biopsy the pathognomonic architecture can be missed.
-Amy


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Amy O'Connell, MD, PhD
Allergy/Immunology Fellow
Boston Children's Hospital
Phone: 617-355-6117
amy.oconnell at childrens.harvard.edu

From: Hillary Hernandez-Trujillo <hshernandez at ctallergy.net<mailto:hshernandez at ctallergy.net>>
Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Date: Tuesday, August 20, 2013 11:10 PM
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Subject: [cis-pidd] 2 yo w/increased double negative t-cells and mesenteric adenopathy


I am looking for suggestions if there are other immunodeficiencies we should look for (aside from ALPS) in a patient with increased double negative T-cells and mesenteric adenopathy or if this sounds like it might not be an immune problem at all.


2 year old female with several months of abdominal pain and intra-abdominal lymphadenopathy. She was recently admitted for severe pain, inability to eat, 17 days of vomiting, and hematochezia. Her abdominal MRI showed significant mesenteric adenopathy with some lymph nodes > 2 cm diameter. They appear to be matted around the mesenteric artery thus revealing a possible etiology for her symptoms. She has no cytopenia or splenomegaly. She has had 2.4 to 5% double negative alpha/beta T-cells in peripheral blood and her Vitamin B12 level is 1898. Her lymph node biopsy is not classic for ALPS as she has apoptosis seen on histopathology and her paracortex is not massively enlarged. She does have a mild increase in germinal centers.  Ferritin and fibrinogen both normal with only a slight increase in triglycerides.  LDH 364.  Her ALPS panel sent to Cincinnati Children’s was not consistent with ALPS- TCR a/b DNTs of 2.4%, but lacked greater than 60% B220 TCR a/b DNTCs, CD3+CD25+/HLA DR ratio < 1.0 or CD27+ C cells < 15%.  Oncology has evaluated her with a normal AFP and beta HCG. They do not think her labs or evaluation is consistent with malignancy.


She has been home from the hospital for 10 days, still with dry heaving and gagging.  She tolerates liquids and pureed/soft foods given very slowly throughout the entire day.  For weeks she has been having profuse sweating episodes where she will be dripping.


Immune work up when she was 18 months old due to repeated ear and sinus infections showed a low normal IgG (460), normal IgA and IgM, normal CD3 count, normal CD4 count, normal CD8 count, low normal NK count (136), protective Rubeola and tetanus IgG, low Varicella IgG, and 7 out of 14 strep pneumo serotypes > 1.3. She had a rash consistent with chicken pox in a patch on her leg about a week after vaccination- unclear if this was at the injection site or not.  Her IgG now is 806, IgM 93, IgA 78, IgE 690. Lymphocyte mitogen stimulation studies are pending.


Additional past medical history- she has already had her adenoids removed twice.  She has 2 older siblings- 5yrs and 8yrs who have also had their adenoids removed 3 to 4 times each.  Both older sibs are also prone to ear and sinus infections.


Norovius and rotavirus- negative
Stool culture- Negative for Salmonella, Shigella, Campylobacter and E.coli 0157
Tissue culture- negative
Fungal culture- negative
AFB culture/smear from lymph node- preliminary negative
CMV PCR- negative
C. diff- negative
ESR normal
CRP elevated at 5.6
C3- 105
C4- 15
Eosinophils increased at 12.5% during recent admission (normal in past)


She will be seeing Infectious Disease in follow up to rule out any additional infectious causes.  We are also sending her to Rheumatology for an evaluation as well.



Thank you in advance for your thoughts and comments!



Hillary

Hillary Hernandez-Trujillo, MD
Clinical Assistant Professor
Department of Pediatrics
University of Connecticut School of Medicine
Division of Infectious Diseases and Immunology
Connecticut Children's Medical Center



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