[CIS PIDD] Chronic EBV ?

[Cooper, Megan]

Hi Elias,

As you think about treatment for this patient, I would focus on trying to get at the underlying diagnosis.  Several pneumonias per year with recurrent bronchitis and sinusitis really suggests a more broad defect than just chronic EBV.  An absent tetanus at the age of 6 is concerning if she was immunized at her 5 year check-up – even though she did respond to re-immunization, an undetectable level is unusual and suggests she isn’t forming lasting immunity.

You might consider a PI3K pathway defect and other “CVID-like” disorders with chronic viral infecitons.

Megan


Megan A. Cooper, MD, PhD
Assistant Professor, Department of Pediatrics
Division of Rheumatology
Washington University School of Medicine
Cooper_m at kids.wustl.edu<mailto:Cooper_m at kids.wustl.edu>
Lab website: http://research.peds.wustl.edu/Default.aspx?alias=research.peds.wustl.edu/Labs/Cooper_M


From: Elias Akl MD [mailto:eliasgakl at gmail.com]
Sent: Friday, March 13, 2015 10:14 AM
To: CIS-PIDD
Subject: Chronic EBV ?

Dear all,

Any insights on this patient would be greatly appreciated.

Our patient is a 6 year old Caucasian female with a history of asthma on Budesonide 80 mcg 2 puffs BID and montelukast 4 mg, history of allergic rhinitis on Azelastin nasal spray and cetirizine 5 mg daily presenting for persistent EBV infection. In March of 2013 she was diagnosed with an acute EBV infection for which she received supporting treatment at home. At the time she complained of generalized fatigue, malaise and myalgia along with mild grade fever, headache and occasional abdominal pain. Her symptoms resolved in around a week time and she went back to her normal daily activities.
3 months ago, the patient started again complaining of the same symptoms she had with her EBV infection consisting mainly of daily low grade fevers of 99-100.8 F at around 4 pm, daily frontal headaches that would get worse throughout the day and are relieved by acetaminophen. Her HA were not associated with an aura, were localized and had no accompanying symptoms of nausea or vomiting. She was not sensitive to light or noise and had no seizure like activity. Other symptoms included an almost once a week severe periumbilical pain, stabbing in nature, lasting around 1 hour with no associated symptoms of nausea or vomiting. Patient had an US of the abdomen done with no abnormalities noted. She did also admit to petechial-like spots that would show up once a week on random body parts mainly on her neck, upper back, arms and stomach, last around couple days and then resolve. These lesions show up on average once a week.  Over the last 3 weeks, she had been also complaining of increased itching and had an episode of hives that lasted around 3 days. Other symptoms include general malaise and fatigue although she slept for 11 or 12 hours a day.
On ROS pt had a dry cough that was worse at night, relieved by albuterol with NO SOB. No joint pain, no muscle pain, no joint swelling or decreased range of motion, no lower extremity swelling and no weight loss. Appetite was at baseline and no other household members had the same symptoms.


Patient also describes recurrent infections:

PNA once or twice a year. Last episode was 2 weeks prior to her symptoms.

Bronchitis once or twice a year.

Several sinus infections. Last one almost 2 months ago.

Has been on antibiotics and prednisone on several occasions.

 Patient was up to date on her vaccinations and her physical exam was benign with a 2/6 nonradiating systolic murmur on the pulmonary area of auscultation. She also had petechiae on her neck.

 Labs:

3/2013:

EBV Ab VCA IgM POSITIVE

EBV Early Ag Ab, IgG: NEGATIVE

EBV Ab VCA IgG : NEGATIVE

EBV Ab VCA : NEGATIVE

EBV Nuclear Ag Ab IgG: NEGATIVE

10/2014:

EBV Ab VCA IgM 87.5 U/mL

EBV Early Ag Ab, IgG < 9 U/mL

EBV Ab VCA IgG 94.4 U/mL

EBV Nuclear Ag Ab IgG 412 U/mL

EBV DNA Quant (serum): UNDETECTABLE

EBV DNA Quant (whole blood): POSTIVE.

 11/2014:

IgA 80mg/dL


IgE 79 IU/mL


IgG 648 mg/dL

IgM 75 mg/dL

CRP and ESR Normal


EhrlichChaf IgG/IgM NEGATVE

HGE IgG/IgM NEGATIVE

Toxo and H Influenza NEGATIVE.

LEP within normal limit

CBC with diff WNL

Pneumo titers were positive to only 4/14 and repeat 6 weeks after prevnar was positive for 13/14

Tetanus IgG Ab < 0.10 IU/mL with repeat 1.80 after vaccination.

Questions:
1. Are her symptoms consistent with chronic EBV infection ?
2. What treatment (if any) has worked for this condition?
3. Use of rituximab was thought of as a treatment but she doesn't have severe enough symptoms to justify use of this medication, with its associated long term immune suppression ?


--
Best Regards,

Elias Akl MD
Allergy and Immunology Fellow
Virginia Commonwealth University Health Center
Work email: eakl at mcvh-edu.lb<mailto:eakl at mcvh-edu.lb>
Office: 804-828-8681
Cell: 716-904-3649

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